Code Preferred Term Synonyms Definition Neoplastic Status C7419 Acanthoma A benign skin neoplasm composed of epithelial cells. Benign C3173 Accelerated Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive Accelerated Phase CML || Accelerated Phase Chronic Granulocytic Leukemia || Accelerated Phase Chronic Myelocytic Leukemia || Accelerated Phase Chronic Myelogenous Leukemia || Accelerated Phase Chronic Myeloid Leukemia || Aggressive-Phase CML || Aggressive-Phase Chronic Myelocytic Leukemia || Aggressive-Phase Chronic Myelogenous Leukemia || Aggressive-Phase Chronic Myeloid Leukemia A phase of chronic myelogenous leukemia characterized by one or more of the following: 1) Myeloblasts accounting for 10-19% of the peripheral blood white cells or of the nucleated cells in the bone marrow, 2) peripheral blood basophils at least 20%, 3) persistent thrombocytopenia that is unrelated to therapy, 4) persistent thrombocytosis despite adequate therapy, 5) increasing white blood cell count and increasing spleen size unresponsive to therapy, and/or evidence of clonal evolution. (WHO, 2001) Malignant C45929 Acidophil Stem Cell Pituitary Gland Adenoma Acidophil Stem Cell Adenoma An infrequent pituitary gland adenoma composed of cells showing immunoreactivity for prolactin and less often growth hormone. Electron microscopic studies reveal giant mitochondria, a finding not present in any other type of pituitary gland adenomas. Benign C3768 Acinar Cell Carcinoma Acinar Adenocarcinoma || Acinar Carcinoma || Acinar Cell Adenocarcinoma || Acinic Cell Adenocarcinoma || Acinic Cell Carcinoma A malignant glandular epithelial neoplasm consisting of secretory cells forming acinar patterns. Representative examples include the acinar cell carcinoma of the pancreas and the acinar adenocarcinoma of the prostate gland. Malignant C27018 Acquired Hemangioma A hemangioma that is not present at birth but develops later in life. Benign C5964 ACTH-Producing Pituitary Gland Carcinoma ACTH Producing Pituitary Gland Carcinoma || Malignant ACTH Producing Neoplasm of Pituitary || Malignant ACTH Producing Neoplasm of Pituitary Gland || Malignant ACTH Producing Neoplasm of the Pituitary || Malignant ACTH Producing Neoplasm of the Pituitary Gland || Malignant ACTH Producing Pituitary Gland Neoplasm || Malignant ACTH Producing Pituitary Gland Tumor || Malignant ACTH Producing Pituitary Neoplasm || Malignant ACTH Producing Pituitary Tumor || Malignant ACTH Producing Tumor of Pituitary || Malignant ACTH Producing Tumor of Pituitary Gland || Malignant ACTH Producing Tumor of the Pituitary || Malignant ACTH Producing Tumor of the Pituitary Gland || Malignant ACTH Secreting Neoplasm of Pituitary || Malignant ACTH Secreting Neoplasm of Pituitary Gland || Malignant ACTH Secreting Neoplasm of the Pituitary || Malignant ACTH Secreting Neoplasm of the Pituitary Gland || Malignant ACTH Secreting Pituitary Gland Tumor || Malignant ACTH Secreting Pituitary Neoplasm || Malignant ACTH Secreting Pituitary Tumor || Malignant ACTH Secreting Tumor of Pituitary || Malignant ACTH Secreting Tumor of Pituitary Gland || Malignant ACTH Secreting Tumor of the Pituitary || Malignant ACTH Secreting Tumor of the Pituitary Gland || Malignant Adrenocorticotropin Producing Neoplasm of Pituitary || Malignant Adrenocorticotropin Producing Neoplasm of Pituitary Gland || Malignant Adrenocorticotropin Producing Neoplasm of the Pituitary || Malignant Adrenocorticotropin Producing Neoplasm of the Pituitary Gland || Malignant Adrenocorticotropin Producing Pituitary Gland Neoplasm || Malignant Adrenocorticotropin Producing Pituitary Gland Tumor || Malignant Adrenocorticotropin Producing Pituitary Neoplasm || Malignant Adrenocorticotropin Producing Pituitary Tumor || Malignant Adrenocorticotropin Producing Tumor of Pituitary || Malignant Adrenocorticotropin Producing Tumor of Pituitary Gland || Malignant Adrenocorticotropin Producing Tumor of the Pituitary || Malignant Adrenocorticotropin Producing Tumor of the Pituitary Gland || Malignant Adrenocorticotropin Secreting Pituitary Gland Neoplasm || Malignant Adrenocorticotropin Secreting Pituitary Gland Tumor || Malignant Adrenocorticotropin Secreting Pituitary Neoplasm || Malignant Adrenocorticotropin Secreting Pituitary Tumor || Malignant Corticotropin Secreting Pituitary Gland Neoplasm || Malignant Corticotropinoma of Pituitary || Malignant Corticotropinoma of Pituitary Gland || Malignant Corticotropinoma of the Pituitary || Malignant Corticotropinoma of the Pituitary Gland || Malignant Pituitary Corticotropinoma || Malignant Pituitary Gland Corticotropinoma A rare, hormonally functioning or non-functioning pituitary gland adenocarcinoma that produces corticotropin. It may be associated with Cushing disease. Malignant C3148 Actinic Keratosis Actinic (Solar) Keratosis || Solar Keratosis A precancerous lesion of the skin composed of atypical keratinocytes. It is characterized by the presence of thick, scaly patches of skin. Several histologic variants have been described, including atrophic, acantholytic, and hyperkeratotic variants. Undetermined C3164 Acute Basophilic Leukemia Basophilic Leukemia || Leukemia Basophilic A rare acute myeloid leukemia in which the immature cells differentiate towards basophils. Malignant C6923 Acute Bilineal Leukemia An acute leukemia of ambiguous lineage in which there is a dual population of blasts with each population expressing markers of a distinct lineage (myeloid and lymphoid or B-and T-lymphocyte). (WHO, 2001) Malignant C4673 Acute Biphenotypic Leukemia An acute leukemia of ambiguous lineage characterized by blasts which coexpress myeloid and T or B lineage antigens or concurrent B and T lineage antigens. (WHO, 2001) Malignant C8923 Acute Erythroid Leukemia Acute Erythroblastic Leukemia || Erythroblastic Leukemia || M6 Acute Myeloid Leukemia An acute myeloid leukemia characterized by a predominant immature erythroid population. There are two subtypes recognized: erythroleukemia and pure erythroid leukemia. (WHO, 2001) Malignant C7464 Acute Leukemia of Ambiguous Lineage Acute Leukemia of Indeterminate Lineage An acute leukemia in which the blasts lack sufficient evidence to classify as myeloid or lymphoid or they have morphologic and/or immunophenotypic characteristics of both myeloid and lymphoid cells. (WHO, 2001) Malignant C3167 Acute Lymphoblastic Leukemia Lymphoblastic Leukemia || Precursor Cell Lymphoblastic Leukemia || Precursor Lymphoblastic Leukemia Leukemia with an acute onset, characterized by the presence of lymphoblasts in the bone marrow and the peripheral blood. It includes the acute B lymphoblastic leukemia and acute T lymphoblastic leukemia. Malignant C121973 Acute Lymphoblastic Leukemia by Gene Expression Profile ALL Gene Expression Cluster Subtype || ALL Gene Expression Subtype || ALL by Gene Expression Profile || ALL by Gene Expression Signature || Acute Lymphoblastic Leukemia Gene Expression Subtype || Acute Lymphoblastic Leukemia by Gene Expression Signature Gene expression-based patient cluster groups in acute lymphoblastic leukemia. Malignant C121978 Acute Lymphoblastic Leukemia by ROSE Cluster ALL Gene Expression Cluster || ALL ROSE Cluster || Acute Lymphoblastic Leukemia Gene Expression Cluster || Acute Lymphoblastic Leukemia ROSE Cluster Unique gene expression-based patient cluster groups in high-risk B-precursor acute lymphoblastic leukemia determined by Recognition of Outliers by Sampling Ends (ROSE). Malignant C3170 Acute Megakaryoblastic Leukemia Acute M7 Myeloid Leukemia || Acute Megakaryoblastic Leukemia (FAB Type M7) || Acute Megakaryocytic Leukemia || FAB M7 An acute myeloid leukemia in which at least 50% of the blasts are of megakaryocytic lineage. (WHO, 2001) Malignant C7318 Acute Monoblastic and Monocytic Leukemia Acute Monoblastic Leukemia and Acute Monocytic Leukemia || Acute Myeloid Leukemia M5 Acute myeloid leukemia in which 80% or more of the leukemic cells are of monocytic lineage, including monoblasts, promonocytes, and monocytes. Bleeding disorders are common presenting features. Malignant C7171 Acute Monoblastic Leukemia Acute Monoblastic Leukemia (FAB M5a) An acute myeloid leukemia in which the monoblasts represent 80% or more of the total cellular population. (WHO, 2001) Malignant C4861 Acute Monocytic Leukemia Acute Monocytic Leukemia (FAB M5b) || Monocytic Leukemia An acute myeloid leukemia in which the majority of monocytic cells are promonocytes. (WHO, 2001) Malignant C3171 Acute Myeloid Leukemia AML - Acute Myeloid Leukemia || Acute Myeloblastic Leukemia || Acute Myelogenous Leukemia || Acute Myelogenous Leukemias || Hematopoeitic - Acute Myleogenous Leukemia (AML) A clonal expansion of myeloid blasts in the bone marrow, blood or other tissues. The classification of acute myeloid leukemias (AMLs) encompasses four major categories: 1) AML with recurrent genetic abnormalities; 2) AML with multilineage dysplasia; 3) Therapy-related AML; 4) AML not otherwise specified. The required bone marrow or peripheral blood blast percentage for the diagnosis of AML is 20% (WHO classification). Malignant C82427 Acute Myeloid Leukemia (Megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 An acute myeloid leukemia associated with t(1;22)(p13.3;q13.1) resulting in the expression of RBM15-MKL1 fusion protein. It affects infants and children and usually shows megakaryocytic maturation. Malignant C4037 Acute Myeloid Leukemia Arising from Previous Myelodysplastic Syndrome AML/MDS || Acute Myeloid Leukemia with Multilineage Dysplasia following Myelodysplastic Syndrome An acute myeloid leukemia developing in patients with a prior history of myelodysplastic syndrome. Malignant C27753 Acute Myeloid Leukemia Not Otherwise Specified Acute Myeloid Leukemia NOS || Acute Myeloid Leukemia Not Otherwise Categorized || Acute Myeloid Leukemia, NOS Acute myeloid leukemias that do not fulfill the criteria for inclusion in the group of acute myeloid leukemias which have recurrent genetic abnormalities or myelodysplastic changes, or are therapy-related. This category includes entities classified according to the French-American-British classification scheme. Malignant C36055 Acute Myeloid Leukemia with a Variant RARA Translocation A term referring to acute myeloid leukemias which show variant translocations involving the RARA gene. The variant fusion partners include NUMA1, ZBTB16, STAT5B, and NPM1. These leukemias often have the morphologic features of acute promyelocytic leukemia. Malignant C82430 Acute Myeloid Leukemia with Gene Mutations AML with Gene Mutations A term referring to acute myeloid leukemias associated with gene mutations most often of the FLT3, NPM1, and CEBPA genes. These leukemias may have normal karyotypes or additional chromosomal aberrations. Malignant C9287 Acute Myeloid Leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 Acute Myeloid Leukemia with Abnormal Marrow Eosinophils || Acute Myeloid Leukemia, CBF-beta/MYH11 || Acute Myeloid Leukemia, CBFB-MYH11 An acute myeloid leukemia with monocytic and granulocytic differentiation and the presence of a characteristically abnormal eosinophil component in the bone marrow. This type of acute myeloid leukemia has a favorable prognosis. (WHO, 2001) Malignant C9018 Acute Myeloid Leukemia with inv(16)(p13.1q22); CBFB-MYH11 Acute Myeloid Leukemia with inv(16)(p13q22) || Acute Myeloid Leukemia with inv(16)(p13q22)(CBFb/MYH11) || Acute Myeloid Leukemia, inv(16)(p13q22) Acute myeloid leukemia characterized by the presence of abnormal bone marrow eosinophils and the characteristic cytogenetic abnormality inv(16)(p13.1q22) which results in the expression of the fusion protein CBFB-MYH11. Malignant C82426 Acute Myeloid Leukemia with inv(3) (q21.3;q26.2) or t(3;3) (q21.3;q26.2); GATA2, MECOM An acute myeloid leukemia associated with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2) resulting in the reposition of a distal GATA2 enhancer to activate MECOM expression. It may present de novo or follow a myelodysplastic syndrome. The clinical course is aggressive. Malignant C122716 Acute Myeloid Leukemia with inv(3) (q21.3;q26.2); GATA2, MECOM Acute Myeloid Leukemia with inv(3)(q21q26.2); RPN1-EVI1 An acute myeloid leukemia associated with inv(3)(q21.3q26.2) resulting in the reposition of a distal GATA2 enhancer to activate MECOM expression. It may present de novo or follow a myelodysplastic syndrome. The clinical course is aggressive. Malignant C3250 Acute Myeloid Leukemia with Maturation AML with Maturation || Acute M2 Myeloid Leukemia || Acute Myeloblastic Leukemia with Maturation || Acute Myelocytic Leukemia with Maturation || Acute Myelogenous Leukemia with Maturation || Acute Myeloid Leukemia (AML-M2) || FAB M2 || M2 Acute Granulocytic Leukemia || M2 Acute Myeloblastic Leukemia || M2 Acute Myeloblastic Leukemia with Maturation || M2 Acute Myelocytic Leukemia with Maturation || M2 Acute Myelogenous Leukemia || M2 Acute Myelogenous Leukemia with Maturation || M2 Acute Myeloid Leukemia || M2 Acute Myeloid Leukemia with Maturation An acute myeloid leukemia (AML) characterized by blasts with evidence of maturation to more mature neutrophils. (WHO, 2001) Malignant C8460 Acute Myeloid Leukemia with Minimal Differentiation AML with Minimal Differentiation || Acute Myeloblastic Leukemia with Minimal Differentiation || Acute Myeloblastic Leukemia, Minimally Differentiated || Acute Myelogenous Leukemia with Minimal Differentiation || Acute Myeloid Leukemia with Minimal Differentiation (MO) || Acute Myeloid Leukemia, Minimally Differentiated || M0 Acute Myeloblastic Leukemia || M0 Acute Myelogenous Leukemia || M0 Acute Myelogenous Leukemia with Minimal Differentiation || M0 Myeloid Leukemia || M0 Myeloid Leukemia with Minimal Differentiation An acute myeloid leukemia (AML) in which the blasts do not show evidence of myeloid differentiation by morphology and conventional cytochemistry. (WHO, 2001) Malignant C9289 Acute Myeloid Leukemia with Multilineage Dysplasia AML with Multilineage Dysplasia || De novo Acute Myeloid Leukemia with Multilineage Dysplasia An acute myeloid leukemia arising de novo and not as a result of treatment. It is characterized by the presence of myelodysplastic features in at least 50% of the cells of at least two hematopoietic cell lines. Patients often present with severe cytopenia. Malignant C82433 Acute Myeloid Leukemia with Mutated CEBPA AML with Mutated CEBPA || Acute Myeloid Leukemia with Non-Germline Mutated CEBPA || Non-Familial Acute Myeloid Leukemia with Mutated CEBPA An acute myeloid leukemia with non-germline mutations of the CEBPA gene. Malignant C82431 Acute Myeloid Leukemia with Mutated NPM1 AML with Mutated NPM1 || Acute Myeloid Leukemia with Cytoplasmic Nucleophosmin An acute myeloid leukemia with mutation of the nucleophosmin gene. It is usually associated with normal karyotype and frequently has myelomonocytic or monocytic features. It usually responds to induction therapy. Malignant C7600 Acute Myeloid Leukemia with Myelodysplasia-Related Changes AML with Myelodysplasia-Related Changes An acute myeloid leukemia with at least 20% blasts in the bone marrow or blood, and either a previous history of myelodysplastic syndrome, multilineage dysplasia or myelodysplastic syndrome-related cytogenetic abnormalities. There is no history of prior cytotoxic therapy for an unrelated disorder, and there is absence of the cytogenetic abnormalities that are present in acute myeloid leukemia with recurrent genetic abnormalities. Malignant C7175 Acute Myeloid Leukemia with Recurrent Genetic Abnormalities Acute Myeloid Leukemia with Balanced Translocations/Inversions A group of acute myeloid leukemias characterized by recurrent genetic abnormalities, mainly balanced translocations. (WHO, 2001) Malignant C36058 Acute Myeloid Leukemia with t(11;17)(q13;q21) Acute Promyelocytic Leukemia with t(11;17)(q13;q21); NUMA1-RARA Acute myeloid leukemia with the variant RARA t(11;17)(q13;q21) and the expression of NUMA1-RARA fusion protein. Malignant C36056 Acute Myeloid Leukemia with t(11;17)(q23;q21) Acute Promyelocytic Leukemia with t(11;17)(q23;q21); PLZF-RARA || Acute Promyelocytic Leukemia with t(11;17)(q23;q21); ZBTB16-RARA Acute myeloid leukemia with the variant RARA t(11;17)(q23;q21) and the expression of PLZF-RARA fusion protein. It lacks differentiation response to retinoids treatment. Malignant C9019 Acute Myeloid Leukemia with t(16;16)(p13.1;q22); CBFB-MYH11 Acute Myeloid Leukemia with t(16;16)(p13;q22) || Acute Myeloid Leukemia, t(16;16)(p13;q22) Acute myeloid leukemia characterized by the presence of abnormal bone marrow eosinophils and the characteristic cytogenetic abnormality t(16;16)(p13.1;q22) which results in the expression of the fusion protein CBFB-MYH11. Malignant C38377 Acute Myeloid Leukemia with t(17;17)(q21;q21) Acute Promyelocytic Leukemia with t(17;17)(q21;q21); STAT5B-RARA Acute myeloid leukemia with the variant RARA t(17;17)(q21;q21) and the expression of STAT5B-RARA fusion protein. Malignant C122717 Acute Myeloid Leukemia with t(3;3) (q21.3;q26.2); GATA2, MECOM Acute Myeloid Leukemia with t(3;3)(q21;q26.2); RPN1-EVI1 An acute myeloid leukemia associated with t(3;3)(q21.3;q26.2) resulting in the reposition of a distal GATA2 enhancer to activate MECOM expression. It may present de novo or follow a myelodysplastic syndrome. The clinical course is aggressive. Malignant C36057 Acute Myeloid Leukemia with t(5;17)(q35;q21) Acute Myeloid Leukemia with t(5;17)(q35;q21);NPM1-RARA Acute myeloid leukemia with the variant RARA t(5;17)(q35;q21) and the expression of NPM1-RARA fusion protein. Malignant C82423 Acute Myeloid Leukemia with t(6;9) (p23;q34.1); DEK-NUP214 Acute Myeloid Leukemia with t(6;9)(p23;q34); DEK-NUP214 An acute myeloid leukemia associated with t(6;9)(p23;q34.1) resulting in DEK-NUP214(CAN) fusion protein expression. It is often associated with multilineage dysplasia and basophilia. It affects both children and adults and it usually has an unfavorable clinical outcome. Malignant C122690 Acute Myeloid Leukemia with t(7;12)(q36;p13); HLXB9-ETV6 Acute Myeloid Leukemia with t(7;12)(q36;p13); MNX1-ETV6 An acute myeloid leukemia in infancy characterized by t(7;12)(q36;p13) and fusion of the homeobox gene HLXB9 (MNX1) with the ETV6 gene. Malignant C9288 Acute Myeloid Leukemia with t(8;21); (q22; q22.1); RUNX1-RUNX1T1 Acute Myeloid Leukemia with t(8;21)(q22;q22) || Acute Myeloid Leukemia with t(8;21)(q22;q22)(AML1(CBFa)/ETO) || Acute Myeloid Leukemia with t(8;21)(q22;q22); RUNX1-RUNX1T1 An acute myeloid leukemia with t(8;21)(q22; q22.1) giving rise to RUNX1/RUNX1T1 fusion transcript and showing maturation in the neutrophil lineage. The bone marrow and the peripheral blood show large myeloblasts with abundant basophilic cytoplasm, often containing azurophilic granules. This type of AML is associated with good response to chemotherapy and high complete remission rate. Malignant C82403 Acute Myeloid Leukemia with t(9;11)(p21.3;q23.3); MLLT3-MLL Acute Myeloid Leukemia with t(9;11)(p21.3;q23.3); MLLT3 (AF9)-KMT2A || Acute Myeloid Leukemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A An acute myeloid leukemia associated with t(9;11)(p21.3;q23.3) and MLLT3-MLL (KMT2A) fusion protein expression. Morphologically it usually has monocytic features. It may present at any age but it is more commonly seen in children. Patients may present with disseminated intravascular coagulation. Malignant C6924 Acute Myeloid Leukemia with Variant MLL Translocations A term referring to acute myeloid leukemias with rearrangement of the MLL gene which results in translocations with various genes other than the MLLT3 (AF9) gene. Malignant C3249 Acute Myeloid Leukemia without Maturation AML without Maturation || Acute M1 Myeloid Leukemia || Acute Myeloblastic Leukemia without Maturation || Acute Myelogenous Leukemia without Maturation || Acute Myeloid Leukemia without Maturation (FAB M1) || FAB M1 || M1 Acute Myeloblastic Leukemia || M1 Acute Myeloblastic Leukemia without Maturation || M1 Acute Myelogenous Leukemia || M1 Acute Myelogenous Leukemia without Maturation || M1 Acute Myeloid Leukemia || M1 Acute Myeloid Leukemia without Maturation An acute myeloid leukemia (AML) characterized by blasts without evidence of maturation to more mature neutrophils. (WHO, 2001) Malignant C7463 Acute Myelomonocytic Leukemia Acute M4 Myeloid Leukemia || Acute Myelomonocytic Leukemia (FAB Type M4) An acute leukemia characterized by the proliferation of both neutrophil and monocyte precursors. (WHO, 2001) Malignant C9020 Acute Myelomonocytic Leukemia with Abnormal Eosinophils AMML Eo || FAB M4Eo Acute myelomonocytic leukemia characterized by the presence of abnormal bone marrow eosinophils. It is associated with inv(16)(p13.1;q22) or t(16;16)(p13.1;q22). It has a favorable prognosis. Malignant C42779 Acute Myelomonocytic Leukemia without Abnormal Eosinophils Acute myelomonocytic leukemia without an abnormal eosinophilic component in the bone marrow. Malignant C4344 Acute Panmyelosis with Myelofibrosis Acute (Malignant) Myelofibrosis || Acute (Malignant) Myelosclerosis || Acute Myelofibrosis || Acute Myelosclerosis || Acute Panmyelosis An acute myeloid leukemia characterized by bone marrow fibrosis without preexisting primary myelofibrosis. Malignant C3182 Acute Promyelocytic Leukemia with PML-RARA AML with t(15;17)(q22;q12) || APML - Acute promyelocytic leukemia || Acute Promyelocytic Leukemia || Acute Promyelocytic Leukemia with t(15;17)(q22;q12); PML-RARA || FAB M3 || Promyelocytic Leukemia An acute myeloid leukemia (AML) in which abnormal promyelocytes predominate. It is characterized by the PML-RARA fusion. There are two variants: the typical and microgranular variant. This AML is particularly sensitive to treatment with all trans-retinoic acid and has a favorable prognosis. (WHO) Malignant C9298 Acute Undifferentiated Leukemia Acute Leukemia Not Otherwise Specified || Acute Leukemia, NOS || Leukemia Stem Cell || Stem Cell Acute Leukemia || Stem Cell Leukemia || Undifferentiated Acute Leukemia A rare acute leukemia of ambiguous lineage in which the blasts do not express markers specific to myeloid or lymphoid lineage. Malignant C7644 Adamantinoma Adamantinoma of Long Bones || Extragnathic Adamantinoma A low grade malignant neoplasm arising from the long bones. The tibia is the most frequently affected bone site. Patients present with swelling which may or may not be associated with pain. Morphologically, it is characterized by a biphasic pattern consisting of an epithelial and an osteofibrous component. The vast majority of cases recur if they are not treated with radical surgery. In a minority of cases the tumor may metastasize to other anatomic sites including lymph nodes, lungs, liver, brain, and skeleton. Malignant C2852 Adenocarcinoma A common cancer characterized by the presence of malignant glandular cells. Morphologically, adenocarcinomas are classified according to the growth pattern (e.g., papillary, alveolar) or according to the secreting product (e.g., mucinous, serous). Representative examples of adenocarcinoma are ductal and lobular breast carcinoma, lung adenocarcinoma, renal cell carcinoma, hepatocellular carcinoma (hepatoma), colon adenocarcinoma, and prostate adenocarcinoma. Malignant C4123 Adenocarcinoma In Situ A lesion in which the normally situated glands are partially or completely replaced by atypical cells with malignant characteristics. Malignant C2970 Adenoid Cystic Carcinoma Adenocystic Carcinoma || Cylindroid Adenocarcinoma A malignant tumor arising from the epithelial cells. Microscopically, the neoplastic epithelial cells form cylindrical spatial configurations (cribriform or classic type of adenoid cystic carcinoma), cordlike structures (tubular type of adenoid cystic carcinoma), or solid structures (basaloid variant of adenoid cystic carcinoma). Adenoid cystic carcinomas mostly occur in the salivary glands. Other primary sites of involvement include the lacrimal gland, the larynx, and the lungs. Adenoid cystic carcinomas spread along nerve sheaths, resulting in severe pain, and they tend to recur. Lymph node metastases are unusual; hematogenous tumor spread is characteristic. Malignant C27413 Adenoma of the Colon with Intramucosal Adenocarcinoma Adenoma of Colon with Intramucosal Adenocarcinoma || Colonic Adenoma with Intramucosal Adenocarcinoma An intramucosal malignant glandular epithelial infiltrate in a colonic adenoma. There is no evidence of submucosal invasion. Malignant C3762 Adenomatoid Tumor Benign Localized Epithelial Mesothelioma || Benign Mesothelial Neoplasm || Benign Mesothelial Tumor || Benign Mesothelioma || Benign Neoplasm of Mesothelium || Benign Neoplasm of the Mesothelium || Benign Tumor of Mesothelium || Benign Tumor of the Mesothelium || Mesothelioma, Benign A benign neoplasm arising from mesothelial cells. It is characterized by the formation of glandular and tubular patterns. It can occur in several anatomic sites including the pleura, peritoneum, and epididymis. Benign C3726 Adenomyoma A benign neoplasm characterized by the presence of a glandular and a mesenchymal (fibromyomatous) component. It occurs in the uterine corpus, cervix, and uterine ligaments. A variant of adenomyoma associated with glandular architectural complexity is called atypical polypoid adenomyoma. Simple polypectomy is usually curative. Atypical polypoid adenomyoma may recur following polypectomy. Benign C9474 Adenosarcoma Mullerian Adenosarcoma || Müllerian Adenosarcoma A low grade malignant neoplasm characterized by the presence of a benign epithelial component (tubular and cleft-like glands) and a low grade sarcomatous component that contains varying amounts of fibrous and smooth muscle tissues. In a minority of cases, the sarcomatous component contains heterologous elements including striated muscle, cartilage, and fat. It occurs in the uterine corpus, ovary, fallopian tube, cervix, and vagina. It may recur and in a minority of cases may metastasize to distant anatomic sites. Malignant C3727 Adenosquamous Carcinoma Mixed Adenocarcinoma and Epidermoid Carcinoma || Mixed Adenocarcinoma and Epidermoid Cell Carcinoma || Mixed Adenocarcinoma and Squamous Carcinoma || Mixed Adenocarcinoma and Squamous Cell Carcinoma An invasive carcinoma composed of malignant glandular cells and malignant squamous cells. Malignant C3775 Adnexal Carcinoma Carcinoma of Adnexa || Carcinoma of Skin Appendage || Skin Appendage Carcinoma A carcinoma arising from the sebaceous glands, sweat glands, or the hair follicles. Representative examples include sebaceous carcinoma, apocrine carcinoma, eccrine carcinoma, and pilomatrical carcinoma. Malignant C9003 Adrenal Cortex Adenoma Adenoma of Adrenal Cortex || Adenoma of Adrenal Gland || Adenoma of the Adrenal Cortex || Adenoma of the Adrenal Gland || Adrenal Adenoma || Adrenal Cortical Adenoma || Adrenal Gland Adenoma || Adrenocortical Adenoma || Benign Adenoma of Adrenal Gland || Benign Adenoma of the Adrenal Gland || Benign Adrenal Adenoma || Benign Adrenal Gland Adenoma A benign neoplasm that can arise from any of the adrenal cortical layers. It can be associated with the overproduction of glucocorticoids (Cushing's syndrome), androgenic or estrogenic steroids (adrenogenital syndrome), or mineralocorticoids (Conn's syndrome). (Sternberg Diagnostic Surgical Pathology, 3rd ed.) Benign C9325 Adrenal Cortex Carcinoma Adrenal Cortex Adenocarcinoma || Adrenal Cortex Cancer || Adrenal Cortical Adenocarcinoma || Adrenal Cortical Carcinoma || Adrenocortical Carcinoma || Carcinoma of Adrenal Cortex || Carcinoma of the Adrenal Cortex A rare, usually large (greater than 5cm), malignant epithelial tumor arising from the adrenal cortical cells. Symptoms are usually related to the excessive production of hormones, and include Cushing's syndrome and virilism in women. Common sites of metastasis include liver, lung, bone, and retroperitoneal lymph nodes. Advanced radiologic procedures have enabled the detection of small tumors, resulting in the improvement of the 5-year survival. Malignant C3326 Adrenal Gland Pheochromocytoma Adrenal Gland Chromaffin Paraganglioma || Adrenal Gland Chromaffinoma || Adrenal Gland Paraganglioma || Adrenal Medullary Paraganglioma || Adrenal Medullary Pheochromocytoma || Adrenal Pheochromocytoma || Chromaffin Paraganglioma of the Adrenal Gland || Intraadrenal Paraganglioma || Pheochromocytoma A benign or malignant neuroendocrine neoplasm of the sympathetic nervous system that secretes catecholamines. It arises from the chromaffin cells of the adrenal medulla. Clinical presentation includes headaches, palpitations, chest and abdominal pain, hypertension, fever, and tremor. Microscopically, a characteristic nesting (zellballen) growth pattern is usually seen. Other growth patterns including trabecular pattern may also be present. Undetermined C27922 Aflatoxins-Related Hepatocellular Carcinoma A hepatocellular carcinoma that develops following exposure to aflatoxin. Malignant C8647 Aggressive NK-Cell Leukemia Aggressive NK-Cell Leukemia/Lymphoma || NK Cell Leukemia || NK-Cell Leukemia || Natural Killer Cell Leukemia A rare, highly aggressive, Epstein-Barr virus-associated leukemia, also known as aggressive NK-cell leukemia/lymphoma; it may represent the leukemic counterpart of nasal type extranodal NK/T-cell lymphomas. It affects primarily teenagers and young adults. It is characterized by the systemic proliferation of NK cells in the peripheral blood, bone marrow, liver, and spleen. Malignant C66796 Aggressive Osteoblastoma An osteoblastoma characterized by the presence of prominent epithelioid osteoblasts and recurrences. Undetermined C9285 Aggressive Systemic Mastocytosis Advanced Systemic Mastocytosis An aggressive and progressive mast cell neoplasm characterized by systemic infiltration of internal organs by aggregates of neoplastic mast cells. There is no evidence of mast cell leukemia or clonal hematologic malignancy. Clinical symptoms include hepatomegaly, splenomegaly, portal hypertension, malabsorption syndrome, and pathologic fractures. Malignant C27924 Alcohol-Related Hepatocellular Carcinoma A hepatocellular carcinoma that develops following alcohol-induced injury of the liver parenchyma. Malignant C7225 ALK-Positive Large B-Cell Lymphoma Diffuse Large B-Cell Lymphoma with Expression of Full-Length ALK || Diffuse Large B-Cell Lymphoma with Expression of Full-Length Anaplastic Lymphoma Kinase A usually aggressive large B-cell lymphoma characterized by the presence of monomorphic immunoblast-like neoplastic B-lymphocytes in a sinusoidal growth pattern. The neoplastic B-lymphocytes express the ALK kinase but they lack the 2;5 translocation. Malignant C27754 Alkylating Agent-Related Acute Myeloid Leukemia Alkylating Agent Related Acute Myeloid Leukemia Acute myeloid leukemia occurring as late complication of prior therapy with alkylating agents. Malignant C7643 Alkylating Agent-Related Myelodysplastic Syndrome Alkylating Agent Related Myelodysplastic Syndrome A disorder seen following cancer chemotherapy. It is the most common cause of therapy-related myelodysplastic syndromes. It typically manifests several years after initiation of single or multi-agent chemotherapy with alkylators. Mutagenic potential of alkylating agents is believed to be age and cumulative dose-dependent. Deletions in chromosomes 5 and 7 are associated with susceptibility to this disorder. Clinical signs may include fatigue, dyspnea, bruising and frequent infections. Clinical course may progress to bone marrow failure or acute myeloid leukemia that is refractory to treatment. Prognosis is dismal with survivability usually less than one year. Malignant C3132 Alpha Heavy Chain Disease Immunoproliferative Small Intestinal Disease || Mediterranean Abdominal Lymphoma || Mediterranean Lymphoma A clonal disorder, also known as immunoproliferative small intestinal disease or Mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain. It predominantly affects young people in the Mediterranean region. It involves the small intestine, and patients usually present with malabsorption syndrome, abdominal pain, weight loss, and fever. There is extensive villous atrophy of the small intestinal mucosa, which is heavily infiltrated by small lymphocytes and plasma cells. The small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma). Malignant C4140 Alveolar Adenoma Adenoma of Alveoli || Adenoma of the Alveoli A benign, well circumscribed lung neoplasm morphologically characterized by the presence of cystic spaces resembling alveoli, lined by a simple cuboidal epithelium. The cystic spaces are surrounded by a spindle cell stroma which may show myxoid changes. It is a solitary, usually peripheral lung lesion. Patients are usually asymptomatic and its discovery is an incidental finding during chest X-ray examination. Surgical excision is curative. Benign C3749 Alveolar Rhabdomyosarcoma Monomorphous Round Cell Rhabdomyosarcoma A rapidly growing malignant mesenchymal neoplasm. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma resembling an alveolar growth pattern. The tumor usually presents in the extremities. Malignant C3802 Amelanotic Melanoma A melanoma characterized by the complete absence of melanin pigment in the melanoma cells. It occurs more frequently on the face and it is often associated with desmoplastic reaction. Malignant C6650 Ampulla of Vater Adenocarcinoma Adenocarcinoma of Ampulla of Vater || Ampullary Adenocarcinoma || Invasive Adenocarcinoma of the Ampullary Region An invasive carcinoma with glandular differentiation arising from the ampulla of Vater. Signs and symptoms include jaundice, abdominal pain, anorexia, nausea, vomiting, and weight loss. Malignant C6657 Ampulla of Vater Adenoma, Intestinal-Type Ampulla of Vater Adenoma || Ampulla of Vater Intestinal-Type Adenoma || Ampullary Adenoma An adenoma that arises from the ampulla of Vater and resembles the adenomas of the small and large intestines. Morphologically, according to the growth pattern, it may be classified as tubular, tubulovillous, or villous. Dysplasia is always present. Jaundice may be an early symptom. Benign C3908 Ampulla of Vater Carcinoma Ampulla of Vater Cancer || Ampullary Carcinoma A carcinoma arising from the ampulla of Vater. The vast majority of cases are adenocarcinomas. Signs and symptoms include jaundice, abdominal pain, anorexia, nausea, vomiting, and weight loss. Malignant C5600 Anal Adenocarcinoma Adenocarcinoma of Anus || Adenocarcinoma of the Anus An adenocarcinoma arising in the anal canal epithelium, including the mucosal surface, the anal glands, and the lining of fistulous tracts. The prognosis is related to the stage at diagnosis. Malignant C7471 Anal Canal Adenocarcinoma Adenocarcinoma Arising in Anal Mucosa || Adenocarcinoma Arising in the Anal Mucosa || Adenocarcinoma of Anal Canal || Adenocarcinoma of the Anal Canal An anal adenocarcinoma arising from the anal canal mucosa. Morphologically, it resembles the adenocarcinoma which arises from the colorectal glandular epithelium. Symptoms include anal pruritus, discomfort when sitting, pain, change in bowel habit, and bleeding. Malignant C7489 Anal Canal Carcinoma Anal Canal Cancer || Anal Canal and Perianal Gland Cancer || Anal Canal and Perianal Gland Carcinoma A squamous cell carcinoma or less frequently an adenocarcinoma, often associated with human papillomavirus (HPV) infection. Homosexual men are at particular risk. The most important prognostic factors are tumor stage and nodal status. Malignant C7469 Anal Canal Squamous Cell Carcinoma A squamous cell carcinoma arising from the mucosa of the anal canal. Malignant C9291 Anal Carcinoma Anal Cancer || Cancer of Anus || Cancer of the Anus || Carcinoma of Anus || Carcinoma of the Anus A carcinoma that arises from the anus. Anal carcinomas include the anal canal and perianal area (anal margin) carcinomas. Perianal carcinomas are staged separately as skin carcinomas in AJCC v6 and v7 editions. The AJCC v8 edition staging system applies to all carcinomas arising in the anal canal, including carcinomas that arise within anorectal fistulas and those arising in the perianal area (anal margin). Malignant C7474 Anal Extramucosal (Perianal) Adenocarcinoma Extramucosal (Perianal) Adenocarcinoma of Anus || Extramucosal (Perianal) Adenocarcinoma of the Anus An anal adenocarcinoma arising from the lining of an anorectal fistulous tract or the anal glands. The overlying anal mucosa does not show evidence of neoplastic changes. It usually presents as a submucosal tumor. Malignant C7472 Anal Margin Carcinoma Carcinoma of Anal Margin || Carcinoma of the Anal Margin || Perianal Skin Carcinoma A carcinoma arising in the anal margin or perianal skin (below the anal verge and involving the perianal hair-bearing skin). It includes basal cell and squamous cell carcinoma. Anal margin carcinoma is staged as a skin carcinoma, separately from anal canal carcinoma. Malignant C6925 Anal Margin Squamous Cell Carcinoma Perianal Skin Squamous Cell Carcinoma || Squamous Cell Carcinoma of Anal Margin || Squamous Cell Carcinoma of the Anal Margin A squamous cell carcinoma arising from the perianal skin. Malignant C5598 Anal Paget Disease Anal Paget's Disease || Paget Disease of the Anus || Paget's Disease of Anus || Paget's Disease of the Anus A slowly spreading, erythematous eczematoid plaque in the anal region. Histologically, the basal part or whole thickness of the squamous epithelium is infiltrated by large cells with abundant pale cytoplasm and large nuclei. Half of the cases are associated with an internal malignancy, most often a colorectal adenocarcinoma. The other half of the cases, have a high local recurrence rate and they may become invasive (WHO). Malignant C9161 Anal Squamous Cell Carcinoma Epidermoid Anal Carcinoma || Epidermoid Carcinoma of Anus || Epidermoid Carcinoma of the Anus || Squamous Cell Anal Carcinoma || Squamous Cell Carcinoma of Anus || Squamous Cell Carcinoma of the Anus A squamous cell carcinoma (SCC) arising from the anal canal or the anal margin (perianal skin). Human papillomavirus is detected in the majority of cases. Homosexual HIV-positive men have an increased risk of developing anal squamous cell carcinoma in comparison to the general male population. Symptoms include anal pruritus, discomfort when sitting, pain, change in bowel habit, and bleeding. The prognosis is generally better for anal margin SCC than for anal canal SCC. Malignant C4051 Anaplastic (Malignant) Meningioma Anaplastic Meningioma || Malignant Meningioma || Meningioma, Malignant A WHO grade III meningioma characterized by the presence of malignant morphologic features, including malignant cytology and a very high mitotic index (20 or more mitoses per ten high power fields). Malignant C68644 Anaplastic Adrenal Cortex Carcinoma An adrenal cortex carcinoma characterized by the presence of necrotic changes, hemorrhage, nuclear atypia, and mitotic activity. Malignant C84347 Anaplastic Astroblastoma An astroblastoma characterized by the presence of high mitotic activity, cytologic atypia, and architectural distortion. Malignant C9477 Anaplastic Astrocytoma Grade III Astrocytic Neoplasm || Grade III Astrocytic Tumor || Grade III Astrocytoma || High Grade Astrocytoma || High-Grade Astrocytoma || Malignant Astrocytoma A diffusely infiltrating, WHO grade III astrocytoma with focal or dispersed anaplasia, and a marked proliferative potential. It may arise from a low-grade astrocytoma, but it can also be diagnosed at first biopsy, without indication of a less malignant precursor lesion. It has an intrinsic tendency for malignant progression to glioblastoma. (WHO) Malignant C129290 Anaplastic Astrocytoma, IDH-Mutant Anaplastic astrocytoma carrying IDH mutations. Malignant C129292 Anaplastic Astrocytoma, Not Otherwise Specified Anaplastic Astrocytoma, NOS Anaplastic astrocytoma in which there is insufficient information on the IDH genes status. Malignant C4049 Anaplastic Ependymoma Anaplastic Ependymal Neoplasm || Anaplastic Ependymal Tumor || Malignant Ependymoma || Undifferentiated Ependymal Neoplasm || Undifferentiated Ependymal Tumor || Undifferentiated Ependymoma || WHO Grade III Ependymal Neoplasm || WHO Grade III Ependymal Tumor A WHO grade III malignant glioma of ependymal origin with accelerated growth and an unfavorable clinical outcome, particularly in children. It is characterized by high mitotic activity, often accompanied by microvascular proliferation and pseudo-palisading necrosis. (Adapted from WHO) Malignant C6952 Anaplastic Kidney Wilms Tumor Anaplastic Renal Wilm's Tumor || Anaplastic Renal Wilms Tumor || Anaplastic Renal Wilms' Tumor Wilms tumor of the kidney characterized by the presence of nuclear anaplasia. Anaplasia is defined by the presence of all of the following: multipolar polyploid mitotic figures, marked nuclear enlargement, and hyperchromasia. When anaplasia is diffuse, it is associated with an unfavorable clinical outcome. Malignant C3720 Anaplastic Large Cell Lymphoma CD30 Positive Anaplastic Large Cell Lymphoma A peripheral (mature) T-cell lymphoma, consisting of usually large anaplastic, CD30 positive cells. The majority of cases are positive for the anaplastic large cell lymphoma (ALK) protein. The most frequently seen genetic alteration is a t(2;5) translocation. Majority of patients present with advanced disease. The most important prognostic indicator is ALK positivity, which has been associated with a favorable prognosis. (WHO, 2001) Malignant C37194 Anaplastic Large Cell Lymphoma, ALK-Negative ALK-Negative Anaplastic Large Cell Lymphoma A T-cell peripheral lymphoma morphologically indistinguishable from anaplastic large cell lymphoma, ALK-positive. It is characterized by the absence of the translocation involving the ALK gene and lacks expression of ALK fusion protein. Malignant C37193 Anaplastic Large Cell Lymphoma, ALK-Positive ALK-Positive Anaplastic Large Cell Lymphoma || ALKoma A T-cell peripheral lymphoma composed of usually large, pleomorphic, CD30 positive T-lymphocytes with abundant cytoplasm characterized by the presence of a translocation involving the ALK gene and expression of ALK fusion protein. Most patients present with peripheral and/or abdominal lymphadenopathy, and often have advanced disease and extranodal involvement. Malignant C6916 Anaplastic Lymphoma Anaplastic Large B-Cell Lymphoma A diffuse large B-cell lymphoma variant characterized by the presence of large round, oval, or polygonal cells with bizarre pleomorphic nuclei resembling Hodgkin or Reed-Sternberg cells. It is unrelated to anaplastic large cell lymphoma which is a T-cell non-Hodgkin lymphoma. Malignant C92625 Anaplastic Medulloblastoma A medulloblastoma characterized by marked nuclear pleomorphism, and high mitotic activity. Malignant C6959 Anaplastic Oligoastrocytoma Anaplastic Mixed Glioma || WHO Grade III Mixed Glioma An oligoastrocytoma characterized by the presence of increased cellularity, nuclear atypia, pleomorphism, and high mitotic activity. Malignant C4326 Anaplastic Oligodendroglioma Malignant Oligodendroglioma || Oligodendroglioma, Malignant || Undifferentiated Oligodendroglioma || WHO Grade III Oligodendroglial Neoplasm || WHO Grade III Oligodendroglial Tumor A WHO grade III oligodendroglioma with focal or diffuse malignant morphologic features (prominent nuclear pleomorphism, mitoses, and increased cellularity). Malignant C129321 Anaplastic Oligodendroglioma, IDH-Mutant and 1p/19q-Codeleted An anaplastic oligodendroglioma carrying IDH gene family mutation and combined whole-arm losses of 1p and 19q (1p/19q codeletion). Malignant C129322 Anaplastic Oligodendroglioma, Not Otherwise Specified Anaplastic Oligodendroglioma, NOS An anaplastic oligodendroglioma in which there is insufficient information on the IDH genes and 1p/19q codeletion status. Malignant C129327 Anaplastic Pleomorphic Xanthoastrocytoma WHO Grade III Pleomorphic Xanthoastrocytoma A WHO grade III pleomorphic xanthoastrocytoma characterized by the presence of five or more mitoses per 10 high-power fields. Necrosis may be present. Patients have shorter survival rates when compared to those with WHO grade II pleomorphic xanthoastrocytoma. Malignant C3799 Angiofibroma Angiofibromatous Hyperplasia || Fibrous Papule || Telangiectatic Fibroma A morphologic variant of fibroma characterized by the presence of numerous dilated vascular channels. Benign C7528 Angioimmunoblastic T-Cell Lymphoma Angioimmunoblastic Lymphadenopathy Type T-Cell Lymphoma A peripheral T-cell lymphoma of mature T follicular helper (TFH) cells characterized by systemic disease and a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules and follicular dendritic cells. EBV-positive cells are nearly always present. It is a clinically aggressive lymphoma and seen mainly in older adults. (WHO 2017) Malignant C3747 Angioleiomyoma Angiomyoma || Vascular Leiomyoma A benign, slow-growing neoplasm that arises from the dermis or subcutaneous tissue. It is characterized by the presence of well-differentiated smooth muscle cells which are arranged around numerous vessels. Benign C4332 Angiomatous Meningioma A WHO grade I meningioma characterized by the presence of small and medium sized vessels that predominate over the meningioma cells. Undetermined C3088 Angiosarcoma A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma. Malignant C4169 Apocrine Carcinoma Apocrine Gland Carcinoma || Carcinoma of Apocrine Gland A carcinoma with apocrine differentiation arising from the sweat glands. It presents as single or multiple nodular lesions which may be ulcerated or hemorrhagic and is usually in the axilla and less often in the anogenital region. It grows in the dermis and infiltrates subcutaneous tissues. It is characterized by the presence of large cells with abundant eosinophilic cytoplasm and large often vesicular nuclei. Most cases are slow growing tumors and have a prolonged course. Malignant C43342 Apocrine Hidrocystoma Apocrine Hydrocystoma A slow-growing, usually solitary, dome-shaped benign sweat gland adenoma, most frequently located on the eyelid. It is characterized by a cystic proliferation of apocrine glands. Surgical excision is curative. Benign C7718 Appendix Adenocarcinoma Adenocarcinoma of Appendix || Adenocarcinoma of the Appendix || Appendiceal Adenocarcinoma A malignant neoplasm arising from the glandular epithelium of the appendix with invasion of the appendiceal wall beyond the muscularis mucosa. The majority of cases are well differentiated adenocarcinomas with mucinous stroma formation. Clinically, it may present as an abdominal mass or with symptoms of acute appendicitis. Patients with chronic ulcerative colitis are at a higher risk in developing appendiceal adenocarcinomas compared to the general population. Malignant C43550 Appendix Adenoma A circumscribed neoplasm arising from the glandular epithelium of the appendix. Morphologically, it is characterized by a proliferation of neoplastic glandular cells and it is associated with dysplasia. According to the growth pattern, it may be classified as tubular, villous, or tubulovillous. The dysplasia is classified as mild, moderate, or severe. Benign C9330 Appendix Carcinoma Appendiceal Cancer || Appendix Cancer || Carcinoma of Appendix || Carcinoma of the Appendix A malignant epithelial neoplasm arising from the appendix. The vast majority of the cases are adenocarcinomas. Malignant C3689 Appendix Goblet Cell Adenocarcinoma Appendix Adenocarcinoid Tumor || Appendix Goblet Cell Carcinoid || Appendix Goblet Cell Carcinoid Tumor || Appendix Mixed Carcinoid-Adenocarcinoma || Goblet Cell Carcinoid Tumor || Mucinous Carcinoid Tumor An invasive mixed adenoneuroendocrine carcinoma of the appendix characterized by a submucosal growth and the presence of neoplastic signet-ring cells resembling goblet cells of the intestine. Malignant C43558 Appendix Mucinous Adenocarcinoma An adenocarcinoma arising from the appendix, characterized by the presence of mucinous stroma formation. Malignant C43554 Appendix Signet Ring Cell Carcinoma Appendix Signet-Ring Cell Carcinoma An adenocarcinoma arising from the appendix, characterized by the presence of signet-ring, mucin-producing malignant cells. The signet-ring cells constitute more than fifty-percent of the malignant cells. Malignant C43555 Appendix Small Cell Neuroendocrine Carcinoma Appendix Small Cell Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the wall of the appendix. It is characterized by the presence of malignant small cells. Malignant C27926 Asbestos-Related Malignant Mesothelioma Malignant mesothelioma occurring in a patient exposed to asbestos. Malignant C7542 Askin Tumor Askin's Tumor || PNET of Thoracopulmonary Region || Peripheral Neuroectodermal Tumor of Thoracopulmonary Region || Small Cell Tumor of Thoracopulmonary Region A primitive neuroectodermal tumor (small round blue cell tumor) of the thorax which can involve the periosteum, thoracic wall and/or pleura though it spares the lung parenchyma. Malignant C4324 Astroblastoma A rare glial neoplasm more commonly found in young adults. It is characterized by tumor cells with characteristics suggestive of an astrocytic origin (positive for GFAP), arranged perivascularly. The cells have broad, non-tapering processes radiating towards a central blood vessel. The biologic behavior of astroblastomas is variable, so no WHO grade has been established, yet. (Adapted from WHO.) Undetermined C6958 Astrocytic Tumor Astrocytic Neoplasm || Astroglioma A glial tumor of the brain or spinal cord showing astrocytic differentiation. It includes the following clinicopathological entities: pilocytic astrocytoma, diffuse astrocytoma, anaplastic astrocytoma, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, and glioblastoma. Undetermined C60781 Astrocytoma A tumor of the brain or spinal cord showing astrocytic differentiation. It includes the following clinicopathological entities: pilocytic astrocytoma, diffuse astrocytoma, anaplastic astrocytoma, pleomorphic xanthoastrocytoma, and subependymal giant cell astrocytoma. Undetermined C6917 Atypical Burkitt/Burkitt-Like Lymphoma Atypical Burkitt's/Burkitt's-Like Lymphoma || Burkitt's-like Lymphoma || Burkitt-like Lymphoma A morphologic variant of Burkitt lymphoma characterized by marked nuclear pleomorphism, abundant apoptotic debris, and the presence of tangible body macrophages. Malignant C72074 Atypical Carcinoid Tumor Malignant Carcinoid Tumor A carcinoid tumor characterized by a high mitotic rate, often associated with the presence of necrosis and nuclear pleomorphism. Undetermined C3519 Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative Atypical CML || Atypical Chronic Myeloid Leukemia || Subacute Myeloid Leukemia A myelodysplastic/myeloproliferative neoplasm characterized by the principal involvement of the neutrophil series with leukocytosis and multilineage dysplasia. The neoplastic cells do not have a Philadelphia chromosome or the BCR/ABL fusion gene. (WHO, 2001) Malignant C6505 Atypical Lipomatous Tumor/Well Differentiated Liposarcoma Atypical Lipomatous Tumor || Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma || Well Differentiated Liposarcoma || Well-Differentiated Liposarcoma A locally aggressive mesenchymal neoplasm composed either entirely or partly of an adipocytic proliferation showing at least focal nuclear atypia in both adipocytes and stromal cells. "Atypical lipomatous tumor" and "well-differentiated liposarcoma" are synonyms describing lesions that are morphologically and genetically identical. Amplification of MDM2 and/or CDK4 is almost always present. (WHO 2020) Undetermined C4723 Atypical Meningioma A WHO grade II meningioma characterized by the presence of brain invasion and an increased mitotic activity, or at least three of the following morphologic features: small cells, high cellularity, prominent nucleoli, lack of architectural pattern, and necrosis. Undetermined C6906 Atypical Teratoid/Rhabdoid Tumor Atypical Teratoid/Rhabdoid Tumor (WHO Grade IV) || CNS Rhabdoid Neoplasm || CNS Rhabdoid Tumor || Central Nervous System Rhabdoid Neoplasm || Central Nervous System Rhabdoid Tumor || Malignant Brain Rhabdoid Neoplasm || Malignant Brain Rhabdoid Tumor || Malignant Rhabdoid Neoplasm of Brain || Malignant Rhabdoid Neoplasm of the Brain || Malignant Rhabdoid Tumor of Brain || Malignant Rhabdoid Tumor of the Brain || Primary Malignant Brain Rhabdoid Neoplasm || Primary Malignant Brain Rhabdoid Tumor || Primary Malignant Rhabdoid Neoplasm of Brain || Primary Malignant Rhabdoid Neoplasm of the Brain || Primary Malignant Rhabdoid Tumor of Brain || Primary Malignant Rhabdoid Tumor of the Brain || Rhabdoid Neoplasm of CNS || Rhabdoid Neoplasm of Central Nervous System || Rhabdoid Neoplasm of the CNS || Rhabdoid Neoplasm of the Central Nervous System || Rhabdoid Tumor of CNS || Rhabdoid Tumor of Central Nervous System || Rhabdoid Tumor of the CNS || Rhabdoid Tumor of the Central Nervous System An aggressive malignant embryonal neoplasm arising from the central nervous system. It is composed of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. Mutations of the SMARCB1 gene or very rarely SMARCA4 (BRG1) gene are present. The vast majority of cases occur in childhood. Symptoms include lethargy, vomiting, cranial nerve palsy, headache, and hemiplegia. Malignant C8644 B Acute Lymphoblastic Leukemia B Cell Precursor Type Acute Leukemia || B-Cell ALL || B-Cell Acute Lymphoblastic Leukemia || B-Cell Lymphoblastic Leukemia || B-Cell Precursor Type Acute Leukemia || B-Cell Type Acute Leukemia || Precursor B-Lymphoblastic Leukemia The most frequent type of acute lymphoblastic leukemia. Approximately 75% of cases occur in children under six years of age. This is a good prognosis leukemia. In the pediatric age group the complete remission rate is approximately 95% and the disease free survival rate is 70%. Approximately 80% of children appear to be cured. In the adult age group the complete remission rate is 60-85%. (WHO, 2001) Malignant C80347 B Acute Lymphoblastic Leukemia with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1) B-Acute Lymphoblastic Leukemia with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1) || B-Cell Acute Lymphoblastic Leukemia with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1) A B-cell acute leukemia characterized by the presence of lymphoblasts that carry a translocation between the E2A gene on chromosome 19 and the PBX1 gene on chromosome 1. It occurs in children and less often in adults. Malignant C80343 B Acute Lymphoblastic Leukemia with t(12;21)(p13.2;q22.1); ETV6-RUNX1 B Acute Lymphoblastic Leukemia with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) || B-Acute Lymphoblastic Leukemia with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) || B-Cell Acute Lymphoblastic Leukemia with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) A B-cell acute leukemia characterized by the presence of lymphoblasts that carry a translocation between the TEL gene on chromosome 12 and the AML1 gene on chromosome 21, (p13.2;q22.1). It results in the production of the TEL-AML1 (ETV6-RUNX1) fusion protein. It is commonly seen in children and rarely in adults. It has a favorable clinical outcome. Malignant C80346 B Acute Lymphoblastic Leukemia with t(5;14)(q31.1;q32.3); IL3-IGH B Acute Lymphoblastic Leukemia with t(5;14)(q31;q32); IL3-IGH || B-Acute Lymphoblastic Leukemia with t(5;14)(q31;q32); IL3-IGH || B-Cell Acute Lymphoblastic Leukemia with t(5;14)(q31;q32); IL3-IGH A rare B-cell acute leukemia characterized by the presence of lymphoblasts that carry a translocation between the IL3 gene on chromosome 5 and the IGH locus on chromosome 14, (q31.1;q32.3). It results in eosinophilia. Malignant C36312 B Acute Lymphoblastic Leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1 Acute Lymphoblastic Leukemia, Philadelphia Chromosome Positive || B Acute Lymphoblastic Leukemia with t(9;22)(q34;q11.2); BCR-ABL1 || B-Cell Acute Lymphoblastic Leukemia with t(9;22)(q34;q11.2); BCR-ABL1 || Philadelphia Positive Acute Lymphoblastic Leukemia || Philadelphia Positive Precursor Lymphoblastic Leukemia A B-cell acute leukemia characterized by the presence of lymphoblasts that carry a translocation between the BCR gene on chromosome 22 and the ABL1 gene on chromosome 9. It results in the production of the p190 kd or p210 kd fusion protein. It has an unfavorable clinical outcome. Malignant C80342 B Acute Lymphoblastic Leukemia with t(v;11q23.3); MLL Rearranged B Acute Lymphoblastic Leukemia with t(v;11q23.3); KMT2A Rearranged || B-Acute Lymphoblastic Leukemia with t(v;11q23); MLL Rearranged || B-Cell Acute Lymphoblastic Leukemia with t(v;11q23); MLL Rearranged A B-cell acute leukemia characterized by the presence of lymphoblasts that carry a translocation between the MLL (KMT2A) gene at 11q23.3 and another gene partner resulting in the production of an MLL related fusion protein. It is the most commonly seen leukemia in the pediatric population under the age of one year. The prognosis is unfavorable. Malignant C8936 B Lymphoblastic Leukemia/Lymphoma Precursor B Lymphoblastic Leukemia/Lymphoma || Precursor B-Lymphoblastic Lymphoma/Leukemia A neoplasm of lymphoblasts committed to the B-cell lineage, typically composed of small to medium-sized blast cells. When the neoplasm involves predominantly the bone marrow and the peripheral blood, it is called B acute lymphoblastic leukemia. When it involves nodal or extranodal sites, it is called B lymphoblastic lymphoma. (WHO, 2001) Malignant C80335 B Lymphoblastic Leukemia/Lymphoma with Hyperdiploidy B-Lymphoblastic Leukemia/Lymphoma with Hyperdiploidy A precursor lymphoid neoplasm composed of B-lymphoblasts which contain more than 50 and usually less than 66 chromosomes. It has a favorable clinical outcome. Malignant C80338 B Lymphoblastic Leukemia/Lymphoma with Hypodiploidy B-Lymphoblastic Leukemia/Lymphoma with Hypodiploidy A precursor lymphoid neoplasm composed of B-lymphoblasts which contain less than 46 chromosomes. It has an unfavorable clinical outcome. Malignant C80328 B Lymphoblastic Leukemia/Lymphoma with Recurrent Genetic Abnormalities B-Lymphoblastic Leukemia/Lymphoma with Recurrent Genetic Abnormalities The term refers to precursor lymphoid neoplasms which are composed of B-lymphoblasts and characterized by the presence of recurrent genetic abnormalities. Malignant C80341 B Lymphoblastic Leukemia/Lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1) B-Lymphoblastic Leukemia/Lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1) A precursor lymphoid neoplasm which is composed of B-lymphoblasts and carries a translocation between the E2A gene on chromosome 19 and the PBX1 gene on chromosome 1. Malignant C80334 B Lymphoblastic Leukemia/Lymphoma with t(12;21)(p13.2;q22.1); ETV6-RUNX1 B Lymphoblastic Leukemia/Lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) || B-Lymphoblastic Leukemia/Lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) A precursor lymphoid neoplasm which is composed of B-lymphoblasts and carries a translocation between the TEL gene on chromosome 12 and the AML1 gene on chromosome 21, (p13.2;q22.1). It results in the production of the TEL-AML1 (ETV6-RUNX1) fusion protein. It has a favorable clinical outcome. Malignant C80340 B Lymphoblastic Leukemia/Lymphoma with t(5;14)(q31.1;q32.3); IL3-IGH B Lymphoblastic Leukemia/Lymphoma with t(5;14)(q31;q32); IL3-IGH || B-Lymphoblastic Leukemia/Lymphoma with t(5;14)(q31;q32); IL3-IGH A precursor lymphoid neoplasm which is composed of B-lymphoblasts and carries a translocation between the IL3 gene on chromosome 5 and the IGH locus on chromosome 14, (q31.1;q32.3). It results in eosinophilia. Malignant C80331 B Lymphoblastic Leukemia/Lymphoma with t(9;22)(q34.1;q11.2); BCR-ABL1 B Lymphoblastic Leukemia/Lymphoma with t(9;22)(q34;q11.2); BCR-ABL1 || B-Lymphoblastic Leukemia/Lymphoma with t(9;22)(q34;q11.2); BCR-ABL1 A precursor lymphoid neoplasm which is composed of B-lymphoblasts and carries a translocation between the BCR gene on chromosome 22 and the ABL1 gene on chromosome 9. It results in the production of the p190 kd or p210 kd fusion protein. It has an unfavorable clinical outcome. Malignant C80332 B Lymphoblastic Leukemia/Lymphoma with t(v;11q23.3); MLL Rearranged B Lymphoblastic Leukemia/Lymphoma with t(v;11q23.3); KMT2A Rearranged || B-Lymphoblastic Leukemia/Lymphoma with t(v;11q23); MLL Rearranged A precursor lymphoid neoplasm which is composed of B-lymphoblasts and carries a translocation between the MLL (KMT2A) gene at 11q23.3 and another gene partner resulting in the production of an MLL related fusion protein. Malignant C80326 B Lymphoblastic Leukemia/Lymphoma, Not Otherwise Specified B Lymphoblastic Leukemia/Lymphoma, NOS || B-Lymphoblastic Leukemia/Lymphoma, Not Otherwise Specified The term refers to precursor lymphoid neoplasms which are composed of B-lymphoblasts and characterized by the absence of recurrent genetic abnormalities. Malignant C8868 B Lymphoblastic Lymphoma B-Lymphoblastic Lymphoma || Precursor B-Lymphoblastic Lymphoma An uncommon type of lymphoma. It constitutes approximately 10% of cases of lymphoblastic lymphoma. Approximately 75% of cases reported in a literature review involved patients who were less than 18 years of age. The most commonly affected sites are the skin, bone, soft tissue, and lymph nodes. It has a high remission rate with a median survival of approximately 60 months. (WHO, 2001) Malignant C4226 Balloon Cell Nevus An uncommon variant of melanocytic nevus. It presents as a small pigmented skin lesion. It is characterized by the presence of large melanocytes with clear, foamy or finely vacuolated cytoplasm. It may recur if it is not completely excised. Benign C7027 Barrett Adenocarcinoma Adenocarcinoma Arising in Barrett's Mucosa || Barrett's Adenocarcinoma An adenocarcinoma arising from Barrett metaplastic epithelium in the esophagus. There is evidence supporting the idea that the Barrett adenocarcinomas develop through a stepwise progression through intestinal metaplastic epithelium to epithelial dysplasia to malignancy. Adenocarcinomas arising in the setting of Barrett esophagus are typically papillary and/or tubular. In terms of grading, they are well or moderately differentiated adenocarcinomas. Malignant C9055 Bartholin Gland Carcinoma Bartholin's Gland Cancer || Bartholin's Gland Carcinoma || Carcinoma of Bartholin's Gland || Carcinoma of the Bartholin's Gland A carcinoma that arises from the Bartholin gland. It usually affects women over fifty and presents with enlargement of the Bartholin gland. Various histologic subtypes have been identified and include adenocarcinoma, squamous cell carcinoma, adenoid cystic carcinoma, adenosquamous carcinoma, transitional cell carcinoma, and small cell carcinoma. Malignant C53558 Basal-Like Breast Carcinoma Basal-Like Breast Cancer || Basal-Like Subtype of Breast Carcinoma A biologic subset of breast carcinoma defined by high expression of genes characteristic of basal epithelial cells, including KRT5 and KRT17, annexin 8, CX3CL1, and TRIM29, and usually by lack of expression of the estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor 2 (HER2). It is the most common subtype of breast cancer associated with BRCA1 mutations, and is associated with a poor prognosis. Malignant C4121 Basaloid Carcinoma A malignant epithelial neoplasm characterized by the presence of neoplastic cells with hyperchromatic nuclei, small amount of cytoplasm, and peripheral nuclear palisading. Malignant C37869 B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classic Hodgkin Lymphoma B-Cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma || Gray Zone Lymphoma || Hodgkin-Like Anaplastic Large Cell Lymphoma || Large B-Cell Lymphoma with Hodgkin Features A group of lymphomas displaying molecular, morphologic, immunophenotypic, and clinical overlap between classic Hodgkin lymphoma and diffuse large B-cell lymphoma. This term particularly applies to mediastinal lymphomas with overlapping features of mediastinal (thymic) large B-cell lymphoma and classic Hodgkin lymphoma. Malignant C3457 B-Cell Non-Hodgkin Lymphoma B-Cell Non Hodgkin's Lymphoma || B-Cell Non-Hodgkin's Lymphoma || Lymphomas Non-Hodgkin's B-Cell || Non-Hodgkin's B-Cell Lymphoma || Non-Hodgkin's Lymphoma B-Cell The most common type of non-Hodgkin lymphoma. It includes the most frequently seen morphologic variants which are: diffuse large B-cell lymphoma, follicular lymphoma, small lymphocytic lymphoma and marginal zone B-cell lymphoma. Malignant C7182 B-Cell Proliferation of Uncertain Malignant Potential A B-cell lymphoproliferative disorder with uncertain malignant potential. The representative example of this category is lymphomatoid granulomatosis. Uncertain Malignant Potential C4753 B-Cell Prolymphocytic Leukemia B Prolymphocytic Leukemia A malignant B-cell lymphoproliferative process affecting the blood, bone marrow, and spleen. The B-prolymphocytes are medium-sized, round lymphoid cells with prominent nucleoli. The B-prolymphocytes must exceed 55% of lymphoid cells in the blood. Cases of transformed chronic lymphocytic leukemia (CLL) and CLL with increased prolymphocytes are excluded. The prognosis is poor. (WHO, 2001) Malignant C48305 Benign Adrenal Gland Pheochromocytoma A sporadic or familial pheochromocytoma that is confined to the adrenal gland and does not have any metastatic potential. The majority of cases are sporadic, and usually unilateral. Familial cases are usually bilateral. Benign C5196 Benign Breast Phyllodes Tumor Benign Phyllodes Neoplasm of Breast || Benign Phyllodes Neoplasm of the Breast || Benign Phyllodes Tumor of Breast || Benign Phyllodes Tumor of the Breast || Breast Benign Phyllodes Neoplasm A usually unilateral, benign and well circumscribed biphasic neoplasm that arises from the breast. It usually affects middle-aged women. It is characterized by the presence of a double layer of epithelial cells that are arranged in clefts, surrounded by a cellular, monomorphic spindle cell mesenchymal component. Mitoses are rare. Necrotic changes may be present in large tumors. Benign C6751 Benign Extragonadal Germ Cell Tumor Benign Extragonadal Germ Cell Neoplasm A non-metastasizing germ cell tumor that arises from an anatomic site other than the testis or ovary. Benign C53998 Benign Gastrointestinal Stromal Tumor A gastrointestinal stromal tumor that is characterized by a maximum diameter equal or less than 5 cm (gastric localization), or equal or less than 2 cm (intestinal localization) and no more than 5 mitotic figures per 50 high power fields. Benign C6449 Benign Germ Cell Tumor Benign Germ Cell Neoplasm || Benign Neoplasm of Germ Cell || Benign Neoplasm of the Germ Cell || Benign Tumor of Germ Cell || Benign Tumor of the Germ Cell A germ cell tumor without metastatic potential. Benign C6748 Benign Glomus Tumor Benign Glomus Neoplasm A glomus tumor of small size characterized by the absence of nuclear atypia and mitotic activity. Benign C3252 Benign Granular Cell Tumor Benign Granular Cell Myoblastoma || Benign Granular Cell Neoplasm || Myoblastoma A granular cell tumor that is confined to the site of origin, without metastatic potential. Benign C4474 Benign Mixed Tumor of the Skin Benign Mixed Tumor of Skin || Benign Mixed Tumor of Skin (Chondroid Syringoma) || Benign Mixed Tumor of the Skin (Chondroid Syringoma) || Chondroid Syringoma || Mixed Tumor of the Skin (Chondroid Syringoma) A rare, benign, slow-growing and painless neoplasm of sweat glands. It usually arises in the head and neck. It is characterized by the presence of a mesenchymal chondroid stroma, fibrosis, and epithelial structures. Benign C121901 Benign Notochordal Cell Tumor Intraosseous Benign Notochordal Cell Tumor An intraosseous benign tumor of notochord origin that arises in the bones of the base of the skull, vertebral bodies, sacrum or coccyx. It contains vacuolated tumor cells without atypia, but lacks myxoid matrix, necrosis, and lobular architecture, which are features that characterize its malignant counterpart, chordoma. Most lesions are incidental findings. Benign C4516 Benign Ovarian Germ Cell Tumor Benign Germ Cell Neoplasm of Ovary || Benign Germ Cell Neoplasm of the Ovary || Benign Germ Cell Tumor of Ovary || Benign Germ Cell Tumor of the Ovary || Benign Ovarian Germ Cell Neoplasm || Benign Ovarian Germ Cell Neoplasms || Benign Ovarian Germ Cell Tumors || Germ Cell Neoplasms, Ovarian, Benign || Germ Cell Tumors, Ovarian, Benign || Ovarian Germ Cell Neoplasms, Benign || Ovarian Germ Cell Tumors, Benign A non-metastasizing germ cell tumor that arises from the ovary. The vast majority of cases are benign dermoid cysts. Benign C4274 Benign Phyllodes Tumor Benign Cystosarcoma Phyllodes || Benign Phyllodes Neoplasm A benign, circumscribed fibroepithelial neoplasm arising from the breast and rarely the prostate gland. It is characterized by the presence of epithelial structures which are arranged in clefts and by a hypercellular mesenchymal stroma which is organized in leaf-like structures. There is no evidence of cellular atypia or sarcomatous features. Benign C5532 Benign Prostate Phyllodes Tumor Benign Phyllodes Neoplasm of Prostate || Benign Phyllodes Neoplasm of the Prostate || Benign Phyllodes Tumor of Prostate || Benign Phyllodes Tumor of the Prostate || Benign Prostate Phyllodes Neoplasm A rare benign neoplasm that arises from the prostate gland and is characterized by the presence of hyperplastic glands and stroma that contains spindle-shaped cells. Benign C7571 Benign Skin Melanocytic Nevus Benign Melanocytic Nevus || Benign Melanocytic Nevus of Skin || Benign Melanocytic Nevus of the Skin || Benign Mole || Benign Nevus of Skin || Benign Nevus of the Skin || Benign Skin Nevus A benign, circumscribed proliferation of melanocytes in the skin. Variants include the Spitz nevus, halo nevus, blue nevus, and balloon cell nevus. Benign C8287 Bilateral Breast Carcinoma Bilateral Breast Cancer Carcinoma that affects both breasts in a simultaneous or non-simultaneous manner. Malignant C8713 Bilateral Retinoblastoma Retinoblastoma involving both eyes. This occurs in the majority of patients with the inherited variant. A minority of patient with bilateral retinoblastoma were found to have involvement of the pineal gland as well. Malignant C27813 Bile Duct Adenocarcinoma An adenocarcinoma arising from the intrahepatic or extrahepatic bile ducts. Malignant C2942 Bile Duct Adenoma Adenoma of Bile Duct || Adenoma of the Bile Duct || Cholangioadenoma || Cholangioma A benign, well-demarcated polypoid neoplasm arising from the bile duct epithelium. According to the growth pattern, it is classified as tubular, papillary, or tubulopapillary. Adenomas arising from the extrahepatic bile ducts usually produce symptoms related to biliary obstruction. Benign C27814 Bile Duct Carcinoma Bile Duct Cancer A carcinoma arising from the intrahepatic or extrahepatic bile ducts. Malignant C4282 Biphasic Mesothelioma Malignant Biphasic Mesothelioma || Malignant Mixed Mesothelioma || Mixed Mesothelioma A malignant mesothelioma characterized by the presence of epithelioid and sarcomatoid components, with each component representing at least 10% of the tumor. Malignant C4032 Bladder Adenocarcinoma Adenocarcinoma of Bladder || Adenocarcinoma of Urinary Bladder || Adenocarcinoma of the Bladder || Adenocarcinoma of the Urinary Bladder || Urinary Bladder Adenocarcinoma A rare adenocarcinoma arising from metaplastic bladder epithelium. It is frequently associated with long-standing local irritation. The majority of cases originate from the trigone and the posterior wall of the bladder. Malignant C4912 Bladder Carcinoma Bladder Cancer || Cancer of Bladder || Cancer of Urinary Bladder || Cancer of the Bladder || Cancer of the Urinary Bladder || Carcinoma of Bladder || Carcinoma of Urinary Bladder || Carcinoma of the Bladder || Carcinoma of the Urinary Bladder || Urinary Bladder Cancer || Urinary Bladder Carcinoma A carcinoma arising from the bladder epithelium. Approximately 90% of the bladder carcinomas are transitional cell carcinomas. The remainder are squamous cell carcinomas, adenocarcinomas and small cell neuroendocrine carcinomas. Malignant C7383 Bladder Papillary Urothelial Carcinoma Urothelial Papillary Carcinoma of the Bladder An invasive or non-invasive papillary transitional cell carcinoma of the urinary bladder. It is classified as low - or high-grade. Malignant C4031 Bladder Squamous Cell Carcinoma Bladder Epidermoid Carcinoma || Epidermoid Carcinoma of Bladder || Epidermoid Carcinoma of Urinary Bladder || Epidermoid Carcinoma of the Bladder || Epidermoid Carcinoma of the Urinary Bladder || Squamous Cell Carcinoma of Bladder || Squamous Cell Carcinoma of Urinary Bladder || Squamous Cell Carcinoma of the Bladder || Squamous Cell Carcinoma of the Urinary Bladder || Urinary Bladder Epidermoid Carcinoma || Urinary Bladder Squamous Cell Carcinoma A squamous cell carcinoma of the bladder arising from metaplastic epithelium. It represents less than 10% of bladder carcinomas. The exception is the Middle East along the Nile Valley, where it represents the most common form of carcinoma because of the endemic nature of schistosomiasis. Bladder squamous cell carcinoma is often associated with long-standing chronic inflammation of the bladder and usually has a poor prognosis. The diagnosis of squamous cell carcinoma of the bladder should be reserved for those tumors that are predominantly keratin forming. Malignant C39844 Bladder Urachal Urothelial Carcinoma A transitional cell carcinoma of the urinary bladder that arises from the urachal epithelium. Malignant C39851 Bladder Urothelial Carcinoma Bladder Transitional Cell Carcinoma || Transitional Cell Carcinoma of the Urinary Bladder || Urinary Bladder Transitional Cell Carcinoma || Urinary Bladder Urothelial Carcinoma || Urothelial Carcinoma of the Urinary Bladder The most common morphologic subtype of urinary bladder carcinoma (over 90% of cases). It arises from the transitional epithelium. It most often affects males in their sixth and seventh decades of life. Hematuria is the most common symptom at presentation. Pathologic stage is the strongest predictor of survival. Malignant C9110 Blast Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive Blast Phase CML || Blast Phase Chronic Granulocytic Leukemia || Blast Phase Chronic Myelocytic Leukemia || Blast Phase Chronic Myelogenous Leukemia || Blast Phase Chronic Myeloid Leukemia || Blastic Phase CML || Blastic Phase Chronic Granulocytic Leukemia || Blastic Phase Chronic Myelocytic Leukemia || Blastic Phase Chronic Myelogenous Leukemia || Blastic Phase Chronic Myeloid Leukemia An advanced phase of chronic myelogenous leukemia. It is characterized by: 1. the presence of blasts in the peripheral blood or bone marrow that are at least 20% of the peripheral blood white cells or of the nucleated cells in the bone marrow respectively, or 2. an extramedullary proliferation of blasts, and/or 3. when there are large aggregates and clusters of blasts in the bone marrow biopsy specimen (adapted from WHO, 2001). Malignant C9147 Blastema Predominant Kidney Wilms Tumor Blastema Predominant Kidney Adenosarcoma || Blastema Predominant Nephroblastoma || Blastema Predominant Renal Adenosarcoma || Blastema Predominant Renal Wilm's Tumor || Blastema Predominant Renal Wilms Tumor || Blastema Predominant Renal Wilms' Tumor Wilms tumor of the kidney characterized by the predominance of the blastema component. Malignant C7203 Blastic Plasmacytoid Dendritic Cell Neoplasm Agranular CD4+ CD56+ Hematodermic Neoplasm/Tumor || CD4+/CD56+ Hematodermic Neoplasm A clinically aggressive neoplasm derived from the precursors of plasmacytoid dendritic cells (also called professional type I interferon-producing cells or plasmacytoid monocytes), with a high frequency of cutaneous and bone marrow involvement and leukemic dissemination. (WHO 2017) Malignant C7229 Blastoid Variant Mantle Cell Lymphoma Classic Blastoid Variant Mantle Cell Lymphoma An aggressive mantle cell lymphoma characterized by the presence of neoplastic B-lymphocytes resembling lymphoblasts. Malignant C3803 Blue Nevus Blue Nevus of Skin || Blue Nevus of the Skin || Blue Skin Nevus An intradermal nevus characterized by the presence of benign pigmented dendritic spindle-shaped melanocytes. It most frequently occurs in the skin of the distal upper extremities, followed by the lower extremities, scalp, face, and buttocks. It usually presents as a single blue or blue-black papular lesion less than 1cm in diameter. Simple excision is usually curative. Benign C5316 Borderline Breast Phyllodes Tumor Borderline Breast Phyllodes Neoplasm || Borderline Phyllodes Neoplasm of Breast || Borderline Phyllodes Neoplasm of the Breast || Borderline Phyllodes Tumor of Breast || Borderline Phyllodes Tumor of the Breast || Low Grade Malignant Breast Phyllodes Tumor A phyllodes tumor of the breast characterized by the presence of intermediate features and a stroma which usually resembles low grade fibrosarcoma. Uncertain Malignant Potential C7503 Borderline Phyllodes Tumor Borderline Phyllodes Neoplasm A phyllodes tumor with morphologic characteristics which are intermediate between a benign and a malignant phyllodes tumor. The stromal sarcomatous changes are of low grade and are often reminiscent of low grade fibrosarcomas. Uncertain Malignant Potential C45163 Borrelia Burgdoferi-Associated Cutaneous Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Borrelia Burgdoferi-Associated Cutaneous Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from chronically stimulated lymphoid tissue in the skin in response to Borrelia burgdoferi infection. Malignant C8374 Bowenoid Papulosis Dysplastic papular lesions presenting on the genitalia of either sex. The lesions are associated with human papillomavirus infection. The majority of cases have a benign clinical course, although, a small number of cases with malignant transformation have been reported. Undetermined C2907 Brain Neoplasm Brain Neoplasms || Brain Tumor || Neoplasm of Brain || Neoplasm of the Brain || Tumor of Brain || Tumor of the Brain A benign or malignant neoplasm that arises from or metastasizes to the brain. Undetermined C40367 Breast Acinic Cell Carcinoma Acinic Cell Breast Carcinoma A breast adenocarcinoma characterized by the presence of serous (acinic cell) differentiation. Malignant C5214 Breast Adenocarcinoma Adenocarcinoma of Breast || Adenocarcinoma of the Breast || Mammary adenocarcinoma The most common histologic type of breast carcinoma. Representative examples include invasive ductal carcinoma not otherwise specified, ductal carcinoma in situ, inflammatory carcinoma, secretory carcinoma, signet ring cell carcinoma, tubular carcinoma, invasive lobular carcinoma, and lobular carcinoma in situ. Malignant C5130 Breast Adenoid Cystic Carcinoma Adenocystic Breast Carcinoma || Adenocystic Carcinoma of Breast || Adenocystic Carcinoma of the Breast || Adenoid Cystic Breast Carcinoma || Adenoid Cystic Carcinoma of Breast || Adenoid Cystic Carcinoma of the Breast || Mammary Adenocystic Carcinoma An adenoid cystic carcinoma primarily involving the breast. Three morphologic patterns are seen: cribriform, trabecular, and solid. The prognosis is excellent. Malignant C40382 Breast Adenoma A benign, well circumscribed neoplasm that arises from the breast. Representative examples include tubular adenoma and ductal adenoma. Benign C40383 Breast Apocrine Adenoma A rare, benign and well circumscribed neoplasm that arises from the breast. It is characterized by the proliferation of epithelial cells with extensive apocrine metaplasia. Benign C4872 Breast Carcinoma Breast Cancer || Cancer of Breast || Cancer of the Breast || Carcinoma of Breast || Carcinoma of the Breast || Mammary Carcinoma A carcinoma arising from the breast, most commonly the terminal ductal-lobular unit. It is the most common malignant tumor in females. Risk factors include country of birth, family history, menstrual and reproductive history, fibrocystic disease and epithelial hyperplasia, exogenous estrogens, contraceptive agents, and ionizing radiation. The vast majority of breast carcinomas are adenocarcinomas (ductal or lobular). Breast carcinoma spreads by direct invasion, by the lymphatic route, and by the blood vessel route. The most common site of lymph node involvement is the axilla. Malignant C53553 Breast Carcinoma by Gene Expression Profile A header term that includes the following breast carcinoma subtypes determined by gene expression profiling: luminal A breast carcinoma, luminal B breast carcinoma, HER2 positive breast carcinoma, basal-like breast carcinoma, triple-negative breast carcinoma, and normal breast-like subtype of breast carcinoma. Malignant C5194 Breast Complex Fibroadenoma Complex Breast Fibroadenoma || Complex Fibroadenoma || Complex Fibroadenoma of Breast || Complex Fibroadenoma of the Breast A breast fibroadenoma that displays fibrocystic changes including apocrine metaplasia, sclerosing adenosis, and cyst formation. Benign C40384 Breast Ductal Adenoma Ductal Adenoma A benign, well circumscribed neoplasm that is located within the lumen of a duct in the breast parenchyma. It is characterized by the presence of glandular structures at the periphery and fibrous tissue at the center of the tumor. Benign C4017 Breast Ductal Carcinoma Duct Adenocarcinoma || Duct Carcinoma || Ductal Adenocarcinoma || Ductal Breast Carcinoma || Ductal Carcinoma || Ductal Carcinoma of Breast || Ductal Carcinoma of the Breast A breast carcinoma arising from the ducts. While ductal carcinomas can arise at other sites, this term is universally used to refer to carcinomas of the breast. Ductal carcinomas account for about two thirds of all breast cancers. Two types of ductal carcinomas have been described: ductal carcinoma in situ (DCIS) and invasive breast carcinoma of no special type. The latter often spreads to the axillary lymph nodes and other anatomic sites. The two forms of ductal carcinoma often coexist. Malignant C2924 Breast Ductal Carcinoma In Situ Breast Ductal Carcinoma In Situ, NOS || Breast Ductal Carcinoma In Situ, Not Otherwise Specified || Ductal Breast Carcinoma In Situ || Ductal Carcinoma In Situ || Ductal Carcinoma In Situ of Breast || Ductal Carcinoma In Situ of the Breast || Intraductal Breast Carcinoma || Intraductal Carcinoma || Intraductal Carcinoma of Breast || Intraductal Carcinoma of the Breast || Non-Infiltrating Ductal Adenocarcinoma of Breast || Non-Infiltrating Ductal Adenocarcinoma of the Breast || Non-Infiltrating Ductal Breast Adenocarcinoma || Non-Infiltrating Ductal Breast Carcinoma || Non-Infiltrating Ductal Carcinoma of Breast || Non-Infiltrating Ductal Carcinoma of the Breast || Non-Infiltrating Intraductal Adenocarcinoma || Non-Infiltrating Intraductal Adenocarcinoma of Breast || Non-Infiltrating Intraductal Adenocarcinoma of the Breast || Non-Infiltrating Intraductal Breast Adenocarcinoma || Non-Infiltrating Intraductal Carcinoma || Non-Invasive Ductal Adenocarcinoma of Breast || Non-Invasive Ductal Adenocarcinoma of the Breast || Non-Invasive Ductal Breast Adenocarcinoma || Non-Invasive Ductal Breast Carcinoma || Non-Invasive Ductal Carcinoma of Breast || Non-Invasive Ductal Carcinoma of the Breast || Non-Invasive Intraductal Adenocarcinoma of Breast || Non-Invasive Intraductal Adenocarcinoma of the Breast || Non-Invasive Intraductal Breast Adenocarcinoma A breast carcinoma entirely confined to the mammary ducts. It is also known as DCIS. There is no evidence of invasion of the basement membrane. Currently, it is classified into three categories: High-grade DCIS, intermediate-grade DCIS and low-grade DCIS. In this classification the DCIS grade is defined by a combination of nuclear grade, architectural growth pattern and presence of necrosis. The size of the lesion as well as the grade and the clearance margins play a major role in dictating the most appropriate therapy for DCIS. Malignant C3744 Breast Fibroadenoma Fibroadenoma || Fibroadenoma of Breast || Fibroadenoma of the Breast A benign tumor of the breast characterized by the presence of stromal and epithelial elements. It presents as a painless, solitary, slow growing, firm, and mobile mass. It is the most common benign breast lesion. It usually occurs in women of childbearing age. The majority of fibroadenomas do not recur after complete excision. A slightly increased risk of developing cancer within fibroadenomas or in the breast tissue of patients previously treated for fibroadenomas has been reported. Benign C4273 Breast Giant Fibroadenoma Giant Breast Fibroadenoma || Giant Fibroadenoma || Giant Fibroadenoma of Breast || Giant Fibroadenoma of the Breast A breast fibroadenoma characterized by a very large size. This term has also been used as a synonym for juvenile fibroadenoma by some authors. The latter is characterized by epithelial hyperplasia and an increased stromal cellularity. Benign C4001 Breast Inflammatory Carcinoma Inflammatory Breast Cancer || Inflammatory Breast Carcinoma || Inflammatory Carcinoma of Breast || Inflammatory Carcinoma of the Breast || Mastitis Carcinomatosa An advanced, invasive breast adenocarcinoma characterized by the presence of distinct changes in the overlying skin. These changes include diffuse erythema, edema, peau d'orange (skin of an orange) appearance, tenderness, induration, warmth, enlargement, and in some cases a palpable mass. The skin changes are the consequence of lymphatic obstruction from the underlying invasive breast adenocarcinoma. Microscopically, the dermal lymphatics show prominent infiltration by malignant cells. The invasive breast adenocarcinoma is usually of ductal, NOS type. There is not significant inflammatory cell infiltrate present, despite the name of this carcinoma. Malignant C4271 Breast Intracanalicular Fibroadenoma Intracanalicular Breast Fibroadenoma || Intracanalicular Fibroadenoma || Intracanalicular Fibroadenoma of Breast || Intracanalicular Fibroadenoma of the Breast A morphologic variant of breast fibroadenoma without clinical significance. It is characterized by distortion and compression of the ducts by proliferating stromal cells. Benign C4276 Breast Juvenile Fibroadenoma Juvenile Breast Fibroadenoma || Juvenile Fibroadenoma || Juvenile Fibroadenoma of Breast || Juvenile Fibroadenoma of the Breast A breast fibroadenoma characterized by epithelial hyperplasia and an increased stromal cellularity. Benign C3771 Breast Lobular Carcinoma Lobular Adenocarcinoma || Lobular Breast Carcinoma || Lobular Carcinoma || Lobular Carcinoma of Breast || Lobular Carcinoma of the Breast An adenocarcinoma of the breast arising from the lobules. This is a relatively uncommon carcinoma, represents approximately 10% of the breast adenocarcinomas and is often bilateral or multifocal. Malignant C4018 Breast Lobular Carcinoma In Situ Lobular Breast Carcinoma In Situ || Lobular Ca in situ of Breast || Lobular Ca in situ of the Breast || Lobular Carcinoma In Situ of the Breast || Lobular Carcinoma In Situ of the Breast (LCIS) || Lobular Carcinoma in situ || Lobular Carcinoma in situ of Breast || Non-Infiltrating Lobular Breast Carcinoma || Non-Infiltrating Lobular Carcinoma || Non-Infiltrating Lobular Carcinoma of Breast || Non-Infiltrating Lobular Carcinoma of the Breast || Non-Invasive Lobular Breast Carcinoma || Non-Invasive Lobular Carcinoma of Breast || Non-Invasive Lobular Carcinoma of the Breast A non-invasive adenocarcinoma of the breast characterized by a proliferation of monomorphic cells completely filling the lumina. The overall lobular architecture is preserved. It is frequently multifocal (90% in some series) and bilateral. It seldom becomes invasive; however there is an increased risk of infiltrating ductal adenocarcinoma. Malignant C9119 Breast Medullary Carcinoma Infiltrating Medullary Carcinoma of Breast || Infiltrating Medullary Carcinoma of the Breast || Invasive Medullary Breast Carcinoma || Invasive Medullary Carcinoma of Breast || Invasive Medullary Carcinoma of the Breast || Medullary Breast Carcinoma || Medullary Breast Carcinoma with Lymphoid Stroma || Medullary Carcinoma of Breast || Medullary Carcinoma of the Breast An infiltrating breast carcinoma with a relatively favorable prognosis. It is an uncommon carcinoma, accounting for less than 1% of all infiltrating breast carcinomas. It is well circumscribed, with soft cut surface and often of considerable size. Microscopically, the predominant growth pattern is syncytial with broad anastomosing bands or sheets of malignant cells. The malignant cells are round with abundant cytoplasm and vesicular nuclei. The sheets of malignant cells are associated with a marked lymphoplasmacytic infiltrate. Glandular or tubular structures are absent. Malignant C5160 Breast Mixed Ductal and Lobular Carcinoma Ductal and Lobular Carcinoma || Mixed Ductal and Lobular Breast Carcinoma || Mixed Ductal and Lobular Carcinoma || Mixed Ductal and Lobular Carcinoma of Breast || Mixed Ductal and Lobular Carcinoma of the Breast || Mixed Lobular and Ductal Breast Carcinoma || Mixed Lobular and Ductal Carcinoma || Mixed Lobular and Ductal Carcinoma of Breast || Mixed Lobular and Ductal Carcinoma of the Breast A breast carcinoma characterized by the presence of a lobular and a ductal component. The ductal component comprises less than 50 percent of the tumor. Malignant C9131 Breast Mucinous Carcinoma Colloid Breast Carcinoma || Colloid Carcinoma of Breast || Colloid Carcinoma of the Breast || Infiltrating Colloid Breast Carcinoma || Infiltrating Mucinous Breast Carcinoma || Invasive Colloid Breast Carcinoma || Invasive Mucinous Breast Carcinoma || Mucinous Breast Carcinoma || Mucinous Carcinoma of Breast || Mucinous Carcinoma of the Breast An invasive adenocarcinoma of the breast characterized by the presence of islands of small and uniform cells, surrounded by large amounts of mucin. Pure mucinous breast carcinomas generally have a favorable prognosis. Malignant C47857 Breast Paget Disease Mammary Paget's Disease || Paget Disease of the Breast || Paget's Disease of Breast || Paget's Disease of the Breast A malignant neoplasm in which there is infiltration of the skin overlying the breast by neoplastic large cells with abundant pale cytoplasm and large nuclei with prominent nucleoli (Paget cells). It is almost always associated with an intraductal or invasive ductal carcinoma of the breast. The clinical features include focal skin reddening, and eczema. Retraction of the nipple may sometimes occur. Malignant C9134 Breast Papillary Carcinoma Papillary Breast Carcinoma || Papillary Carcinoma of Breast || Papillary Carcinoma of the Breast A breast carcinoma characterized by the formation of irregular, finger-like projections of fibrous stroma covered with neoplastic epithelial cells. Malignant C4190 Breast Papillary Ductal Carcinoma In Situ Breast Intraductal Papillary Carcinoma || Breast Papillary DCIS || Intraductal Papillary Adenocarcinoma || Intraductal Papillary Breast Adenocarcinoma || Intraductal Papillary Breast Carcinoma || Intraductal Papillary Carcinoma || Non-Infiltrating Intraductal Papillary Adenocarcinoma || Non-Infiltrating Intraductal Papillary Carcinoma || Non-Infiltrating Papillary Breast Adenocarcinoma || Non-Infiltrating Papillary Breast Carcinoma || Non-Invasive Intraductal Papillary Adenocarcinoma || Non-Invasive Intraductal Papillary Carcinoma || Non-Invasive Papillary Breast Adenocarcinoma || Non-Invasive Papillary Breast Carcinoma || Papillary DCIS || Papillary Ductal Carcinoma In Situ Breast ductal carcinoma in situ characterized by the presence of filiform arborizing fibrovascular cores lined by neoplastic ductal epithelium. (WHO 2019) Malignant C4272 Breast Pericanalicular Fibroadenoma Pericanalicular Breast Fibroadenoma || Pericanalicular Fibroadenoma || Pericanalicular Fibroadenoma of Breast || Pericanalicular Fibroadenoma of the Breast A morphologic variant of breast fibroadenoma without clinical significance. It is characterized by circumferential proliferation of stromal cells around the ducts. This results in the formation of rounded ductal-epithelial structures. Benign C7575 Breast Phyllodes Tumor Breast Phyllodes Neoplasm || Phyllodes Breast Neoplasm || Phyllodes Breast Tumor || Phyllodes Neoplasm of Breast || Phyllodes Neoplasm of the Breast || Phyllodes Tumor of Breast || Phyllodes Tumor of the Breast A benign, malignant, or borderline circumscribed biphasic neoplasm that arises from the breast. It usually affects middle-aged women. It is characterized by the presence of a double layer of epithelial cells that are arranged in clefts, surrounded by a spindle cell mesenchymal (stromal) component. Undetermined C40408 Breast Pleomorphic Adenoma Pleomorphic Adenoma of the Breast A rare, benign and well circumscribed neoplasm that arises from the breast. It is characterized by the proliferation of epithelial and myoepithelial cells surrounded by chondroid stroma. Benign C4189 Breast Secretory Carcinoma Cystic Hypersecretory Breast Carcinoma || Cystic Hypersecretory Carcinoma of Breast || Cystic Hypersecretory Carcinoma of the Breast || Infiltrating Cystic Hypersecretory Duct Breast Carcinoma || Invasive Cystic Hypersecretory Duct Breast Carcinoma || Juvenile Breast Carcinoma || Juvenile Carcinoma of Breast || Juvenile Carcinoma of the Breast || Juvenile Secretory Breast Carcinoma || Juvenile Secretory Carcinoma of Breast || Juvenile Secretory Carcinoma of the Breast || Secretory Breast Carcinoma || Secretory Carcinoma || Secretory Carcinoma of Breast || Secretory Carcinoma of the Breast A rare, low grade invasive adenocarcinoma of the breast characterized by the presence of cells that secrete milk-like material. Morphologically, it usually appears as a circumscribed lesion, composed of cystic spaces, tubular structures, and solid areas. Malignant C6760 Breast Small Cell Neuroendocrine Carcinoma Breast Small Cell Carcinoma || Small Cell Carcinoma of Breast || Small Cell Carcinoma of the Breast || Small Cell Neuroendocrine Carcinoma of Breast || Small Cell Neuroendocrine Carcinoma of the Breast A poorly differentiated neuroendocrine carcinoma that arises from the breast. It is characterized by the presence of small neuroendocrine cells and high mitotic activity. Malignant C5177 Breast Squamous Cell Carcinoma Primary Squamous Cell Breast Carcinoma || Primary Squamous Cell Carcinoma of Breast || Primary Squamous Cell Carcinoma of the Breast || SCC of Breast || SCC of the Breast || Squamous Breast Carcinoma || Squamous Carcinoma of Breast || Squamous Carcinoma of the Breast || Squamous Cell Breast Carcinoma || Squamous Cell Carcinoma of Breast || Squamous Cell Carcinoma of the Breast A rare carcinoma that arises from the breast parenchyma and is entirely composed of squamous cells. Malignant C62210 Breast Tubular Adenoma A benign, well circumscribed neoplasm that arises from the breast. It is composed entirely of tubular structures that contain epithelial and myoepithelial cells. Benign C9135 Breast Tubular Carcinoma Infiltrating Tubular Breast Carcinoma || Infiltrating Tubular Carcinoma of Breast || Infiltrating Tubular Carcinoma of the Breast || Invasive Tubular Breast Carcinoma || Invasive Tubular Carcinoma of Breast || Invasive Tubular Carcinoma of the Breast || Tubular Breast Carcinoma || Tubular Carcinoma of Breast || Tubular Carcinoma of the Breast An invasive adenocarcinoma of the breast with a favorable prognosis. It is composed of tubular structures lined by a single layer of epithelium. Malignant C5264 Bronchial Mucosa-Associated Lymphoid Tissue Lymphoma BALT Lymphoma || BALToma || Bronchial MALT Lymphoma || Bronchus-Associated Lymphoid Tissue Lymphoma An extranodal marginal zone lymphoma that arises from the lung. It is characterized by the neoplastic proliferation of small B-lymphocytes, monocytoid cells and cells with plasma cell differentiation in the marginal zones of reactive lymphoid follicles. The neoplastic cells infiltrate the interfollicular areas and the bronchial epithelium forming lymphoepithelial lesions. The neoplasm is usually discovered as a mass in a chest x-ray in asymptomatic patients. When symptoms occur, they include cough, dyspnea, hemoptysis, and chest pain. If the lung lesions are resectable, surgery can result in prolonged remission. Malignant C35875 Bronchogenic Carcinoma Bronchial Carcinoma || Bronchiogenic Carcinoma || Bronchogenic Lung Carcinoma A lung carcinoma arising from the bronchial epithelium. Malignant C4040 Buccal Mucosa Squamous Cell Carcinoma SCC of Buccal Mucosa || SCC of the Buccal Mucosa || Squamous Cell Carcinoma of Buccal Mucosa || Squamous Cell Carcinoma of the Buccal Mucosa A squamous cell carcinoma of the oral cavity that arises from the buccal mucosa. Malignant C7400 Burkitt Leukemia Burkitt's Cell Leukemia || Burkitt's Leukemia The leukemic counterpart of Burkitt's lymphoma. The characteristic Burkitt cells are seen in the bone marrow and the peripheral blood. This is an aggressive leukemia. Malignant C2912 Burkitt Lymphoma Burkitt's Lymphoma A highly aggressive lymphoma composed of monomorphic medium-sized B-cells with basophilic cytoplasm and numerous mitotic figures. It is often associated with the presence of Epstein-Barr virus (EBV) and is commonly seen in AIDS patients. Three morphologic variants are recognized: classical Burkitt lymphoma, Burkitt lymphoma with plasmacytoid differentiation, and atypical Burkitt/Burkitt-like lymphoma. All cases express the MYC translocation [t(8;14)]. (WHO, 2001) Malignant C7189 Burkitt Lymphoma with Plasmacytoid Differentiation Burkitt's Lymphoma with Plasmacytoid Differentiation Burkitt lymphoma characterized by the presence of malignant cells with eccentric basophilic cytoplasm. The nucleoli of these cells are often single and central. This morphologic variant of Burkitt lymphoma is more often seen in patients with immunodeficiency. Malignant C7457 Capillary Hemangioma Capillary Angioma A common hemangioma characterized by the presence of capillary-sized vascular channels without prominent epithelioid endothelial cells. Benign C2915 Carcinoid Tumor Carcinoid A slow growing neuroendocrine tumor, composed of uniform, round, or polygonal cells having monotonous, centrally located nuclei and small nucleoli, infrequent mitoses, and no necrosis. The tumor may show a variety of patterns, such as solid, trabecular, and acinar. Electron microscopy shows small secretory granules. Immunohistochemical studies reveal NSE, as well as chromogranin immunoreactivity. Malignant histology (cellular pleomorphism, hyperchromatic nuclei, prominent nucleoli, necrosis, and mitoses) can occasionally be seen. Such cases may have an aggressive clinical course. Gastrointestinal tract and lung are common sites of involvement. Undetermined C2916 Carcinoma Epithelial Carcinoma || Malignant Epithelial Neoplasm || Malignant Epithelial Tumor || Malignant Epithelioma A malignant tumor arising from epithelial cells. Carcinomas that arise from glandular epithelium are called adenocarcinomas, those that arise from squamous epithelium are called squamous cell carcinomas, and those that arise from transitional epithelium are called transitional cell carcinomas. Morphologically, the malignant epithelial cells may display abnormal mitotic figures, anaplasia, and necrosis. Carcinomas are graded by the degree of cellular differentiation as well, moderately, or poorly differentiated. Carcinomas invade the surrounding tissues and tend to metastasize to other anatomic sites. Lung carcinoma, skin carcinoma, breast carcinoma, colon carcinoma, and prostate carcinoma are the most frequently seen carcinomas. Malignant C4397 Carcinoma ex Pleomorphic Adenoma Carcinoma in Pleomorphic Adenoma A carcinoma arising in a pre-existing pleomorphic adenoma. It most often occurs in the parotid gland and less often in the submandibular gland and minor salivary gland. Patients usually present with a history of a long-standing mass which recently had undergone rapid growth. The prognosis depends on the invasiveness of the malignant component. Patients with non-invasive or minimally invasive tumors usually have a good prognosis following surgical resection. Invasive tumors are usually aggressive and are associated with recurrences and metastases. Malignant C2917 Carcinoma In Situ Intraepithelial Carcinoma || Non-invasive Carcinoma A malignant epithelial neoplasm which is confined to the epithelial layer without evidence of further tissue invasion. Malignant C34448 Carcinosarcoma A malignant tumor composed of a mixture of carcinomatous and sarcomatous elements. Malignant C6739 Cardiac Rhabdomyoma Heart Rhabdomyoma || Rhabdomyoma of Heart || Rhabdomyoma of the Heart A well circumscribed benign tumor arising from cardiac muscle. It usually affects children and may be present in the fetus. Depending on tumor location and size, cardiac, respiratory, and hemodynamic parameters may be affected. There is an association between cardiac rhabdomyoma and tuberous sclerosis. Benign C3086 Cavernous Hemangioma Cavernoma || Cavernous Angioma A hemangioma characterized by the presence of cavernous vascular spaces. Benign C53316 Cavernous Lymphangioma A lymphangioma characterized by the presence of thin-walled cavernous lymphatic spaces. Benign C5543 Cecum Adenocarcinoma Adenocarcinoma of Cecum || Adenocarcinoma of the Cecum || Cecal Adenocarcinoma An adenocarcinoma arising from the cecum. It is more frequently seen in populations with a Western type diet and in patients with a history of chronic inflammatory bowel disease. Histologic variants include mucinous adenocarcinoma, signet ring cell carcinoma, medullary carcinoma, serrated adenocarcinoma, cribriform comedo-type adenocarcinoma, and micropapillary adenocarcinoma. Malignant C39815 Cellular Congenital Mesoblastic Nephroma Infantile Renal Fibrosarcoma A congenital mesoblastic nephroma characterized by increased cellularity, sheet-like proliferation of fibroblastic cells, and increased mitotic activity. Necrotic changes are commonly present. Malignant C4713 Cellular Ependymoma An ependymoma which shows conspicuous cellularity without a significant increase in mitotic rate. (Adapted from WHO) Undetermined C6892 Cellular Fibroma A morphologic variant of fibroma characterized by increased cellularity. Benign C5398 Central Nervous System Embryonal Tumor, Not Otherwise Specified CNS Embryonal Tumor, NOS || CNS Embryonal Tumor, Not Otherwise Specified || Central Nervous System Embryonal Tumor, NOS A term that refers to central nervous system embryonal tumors which are not fully characterized. This category includes tumors previously designated as central nervous system primitive neuroectodermal tumors. Malignant C92629 Central Nervous System Ganglioneuroblastoma Cerebral Ganglioneuroblastoma A supratentorial primitive neuroectodermal tumor characterized by the presence of poorly differentiated or undifferentiated neuroepithelial cells and ganglion cells. Malignant C7009 Central Nervous System Germinoma Germinoma of CNS || Germinoma of Central Nervous System || Germinoma of the CNS || Germinoma of the Central Nervous System A malignant germ cell tumor arising from the central nervous system. It is composed of uniform cells resembling primitive germ cells. These cells have large, vesicular nuclei, prominent nucleoli and a clear, glycogen-rich cytoplasm. Additional features are lymphoid or lymphoplasmacytic infiltrates and, less frequently, scattered syncytiotrophoblastic giant cells. (Adapted from WHO) Malignant C7007 Central Nervous System Hemangioblastoma CNS Hemangioblastoma || Central Nervous System Capillary Hemangioblastoma || Hemangioblastoma of CNS || Hemangioblastoma of Central Nervous System || Hemangioblastoma of the CNS A hemangioblastoma arising from structures of the central nervous system. Undetermined C4327 Central Nervous System Medulloepithelioma Medulloepithelioma || Medulloepithelioma, Central Nervous System A rare, usually aggressive malignant embryonal neoplasm of the central nervous system occurring in children. It is characterized by the presence of neuroepithelial cells which form papillary, trabecular, or tubular structures and absence of C19MC amplification. Symptoms include headache, nausea, and vomiting. Malignant C9293 Central Nervous System Neoplasm CNS Neoplasm || CNS Tumor || Central Nervous System Tumor || Neoplasm of CNS || Neoplasm of Central Nervous System || Neoplasm of the Central Nervous System || Tumor of CNS || Tumor of Central Nervous System || Tumor of the CNS || Tumor of the Central Nervous System A benign or malignant, primary or metastatic neoplasm that affects the brain, meninges, or spinal cord. Representative examples of primary neoplasms include astrocytoma, oligodendroglioma, ependymoma, and meningioma. Representative examples of metastatic neoplasms include carcinoma and leukemia. Undetermined C4826 Central Nervous System Neuroblastoma Cerebral Hemispheric Neuroblastoma || Cerebral Neuroblastoma || Cerebral Neuroblastoma, PNET || Neuroblastoma of Cerebral Hemispheres || Neuroblastoma of Cerebrum || Neuroblastoma of the Cerebral Hemispheres || Neuroblastoma of the Cerebrum A neuroblastoma arising from the cerebral hemispheres. Malignant C129526 Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma CNS Solitary Fibrous Tumor/Hemangiopericytoma A mesenchymal, non-meningothelial neoplasm arising from the central nervous system. It is characterized by a collagenous and low cellularity spindle cell and/or hemangiopericytomatous histopathological pattern, recurrent intrachromosomal rearrangement on chromosome 12q that results in the fusion of the NAB2 and STAT6 genes, high recurrence rates, and long-term risk of systemic metastasis. Undetermined C129530 Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma, Grade 1 CNS Solitary Fibrous Tumor/Hemangiopericytoma, Grade 1 || Central Nervous System Solitary Fibrous Tumor A solitary fibrous tumor/hemangiopericytoma that arises from the central nervous system. It corresponds most often to the collagenous and low cellularity spindle cell tumor which was diagnosed as central nervous system solitary fibrous tumor in the past. Undetermined C129528 Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma, Grade 2 CNS Solitary Fibrous Tumor/Hemangiopericytoma, Grade 2 A solitary fibrous tumor/hemangiopericytoma that arises from the central nervous system. It corresponds to the more cellular, less collagenous tumor with plump cells and staghorn vasculature which was diagnosed as central nervous system hemangiopericytoma in the past. Undetermined C129527 Central Nervous System Solitary Fibrous Tumor/Hemangiopericytoma, Grade 3 CNS Solitary Fibrous Tumor/Hemangiopericytoma, Grade 3 A solitary fibrous tumor/hemangiopericytoma that arises from the central nervous system. It most often corresponds to the tumor previously diagnosed as anaplastic hemangiopericytoma. Malignant C3791 Central Neurocytoma Central Neurocytoma (WHO Grade II) An intraventricular neuronal neoplasm composed of uniform round cells with neuronal differentiation. It is typically located in the lateral ventricles in the region of the foramen of Monro. It generally affects young adults and has a favorable prognosis. (Adapted from WHO) Undetermined C4074 Centroblastic Lymphoma Diffuse Centroblastic Lymphoma A B-cell non-Hodgkin lymphoma composed of large noncleaved cells. This is a subtype of diffuse large B-cell non-Hodgkin lymphoma. Malignant C5146 Cerebellar Hemangioblastoma Angioblastoma of Cerebellum || Angioblastoma of the Cerebellum || Cerebellar Angioblastoma || Cerebellar Capillary Hemangioblastoma || Hemangioblastoma of Cerebellum || Hemangioblastoma of the Cerebellum A histologically benign tumor, usually cystic with a vascular mural nodule, that is most often found in the cerebellum though it has been reported at other sites within the neuraxis. It is associated with von Hippel-Lindau disease (VHL gene located on chr 3p25-26). Undetermined C6905 Cerebellar Liponeurocytoma Cerebellar Liponeurocytoma (WHO Grade II) || Lipomatous Medulloblastoma A rare, WHO grade II cerebellar neoplasm which shows consistent neuronal, variable astrocytic and focal lipomatous differentiation. It occurs in adults, has a low proliferative potential and usually has a favorable prognosis. (Adapted from WHO) Undetermined C5436 Cerebellopontine Angle Embryonal Tumor, Not Otherwise Specified Cerebellopontine Angle Embryonal Tumor, NOS A central nervous system embryonal tumor, not otherwise specified arising from the cerebellopontine angle of the infratentorial brain. Malignant C4029 Cervical Adenocarcinoma Adenocarcinoma of Cervix || Adenocarcinoma of Cervix Uteri || Adenocarcinoma of Uterine Cervix || Adenocarcinoma of the Cervix || Adenocarcinoma of the Cervix Uteri || Adenocarcinoma of the Uterine Cervix || Carcinoma of Endocervix || Carcinoma of the Endocervix || Cervix Adenocarcinoma || Cervix Uteri Adenocarcinoma || Endocervical Adenocarcinoma || Endocervical Carcinoma || Uterine Cervix Adenocarcinoma An adenocarcinoma arising from the endocervical glandular epithelium. It is classified as either human papillomavirus-related or human papillomavirus-independent adenocarcinoma. Histologic variants include usual-type, mucinous, mesonephric, serous, clear cell, and endometrioid adenocarcinoma. Malignant C4520 Cervical Adenocarcinoma In Situ Adenocarcinoma in situ of Cervix || Adenocarcinoma in situ of Cervix Uteri || Adenocarcinoma in situ of Uterine Cervix || Adenocarcinoma in situ of the Cervix || Adenocarcinoma in situ of the Cervix Uteri || Adenocarcinoma in situ of the Uterine Cervix || Cervix Adenocarcinoma in situ || Cervix Uteri Adenocarcinoma in situ || Uterine Cervix Adenocarcinoma in situ Stage 0 includes: (Tis, N0, M0). Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. FIGO no longer includes stage 0. (AJCC 7th ed.) Malignant C4519 Cervical Adenosquamous Carcinoma Adenosquamous Cell Carcinoma of Cervix || Adenosquamous Cell Carcinoma of Cervix Uteri || Adenosquamous Cell Carcinoma of Uterine Cervix || Adenosquamous Cell Carcinoma of the Cervix || Adenosquamous Cell Carcinoma of the Cervix Uteri || Adenosquamous Cell Carcinoma of the Uterine Cervix || Cervical Adenosquamous Cell Carcinoma || Cervix Adenosquamous Cell Carcinoma || Cervix Uteri Adenosquamous Cell Carcinoma || Uterine Cervix Adenosquamous Cell Carcinoma An uncommon carcinoma arising from the cervix. It is composed of malignant glandular epithelial cells and malignant squamous epithelial cells. Malignant C9039 Cervical Carcinoma Cancer of Cervix || Cancer of Uterine Cervix || Cancer of the Cervix || Cancer of the Uterine Cervix || Carcinoma of Cervix || Carcinoma of Cervix Uteri || Carcinoma of Uterine Cervix || Carcinoma of the Cervix || Carcinoma of the Cervix Uteri || Carcinoma of the Uterine Cervix || Cervical Cancer || Cervix Cancer || Cervix Carcinoma || Cervix Uteri Carcinoma || Uterine Cervix Cancer || Uterine Cervix Carcinoma A carcinoma arising from either the exocervical squamous epithelium or the endocervical glandular epithelium. The major histologic types of cervical carcinoma are squamous cell carcinoma and adenocarcinoma. Malignant C6856 Cervical Microglandular Polyp Microglandular Polyp of Cervix || Microglandular Polyp of Uterine Cervix || Microglandular Polyp of the Cervix || Microglandular Polyp of the Uterine Cervix || Uterine Cervix Microglandular Polyp A benign endocervical polypoid epithelial hyperplasia characterized by the presence of tightly packed glandular structures. It is usually seen in women on oral contraceptive therapy and during pregnancy. Benign C7982 Cervical Small Cell Neuroendocrine Carcinoma Cervical Small Cell Carcinoma || Cervix Small Cell Carcinoma || Cervix Uteri Small Cell Carcinoma || Small Cell Carcinoma of Cervix || Small Cell Carcinoma of Cervix Uteri || Small Cell Carcinoma of Uterine Cervix || Small Cell Carcinoma of the Cervix || Small Cell Carcinoma of the Cervix Uteri || Small Cell Carcinoma of the Uterine Cervix || Uterine Cervix Small Cell Carcinoma A small cell neuroendocrine carcinoma arising from the cervix. Malignant C4028 Cervical Squamous Cell Carcinoma Cervix Squamous Cell Carcinoma || Cervix Uteri Squamous Cell Carcinoma || Squamous Cell Carcinoma of Cervix || Squamous Cell Carcinoma of Cervix Uteri || Squamous Cell Carcinoma of Uterine Cervix || Squamous Cell Carcinoma of the Cervix || Squamous Cell Carcinoma of the Cervix Uteri || Squamous Cell Carcinoma of the Uterine Cervix || Uterine Cervix Squamous Cell Carcinoma A squamous cell carcinoma arising from the cervical epithelium. It usually evolves from a precancerous cervical lesion. Increased numbers of sexual partners and human papillomavirus (HPV) infection are risk factors for cervical squamous cell carcinoma. Survival is most closely related to the stage of disease at the time of diagnosis. Malignant C7347 Cervical Squamous Cell Carcinoma In Situ Cervical Squamous Cell Carcinoma In Situ AJCC v7 || Cervical Squamous Intraepithelial Neoplasia 3 || Cervix Squamous Cell Carcinoma In Situ || Cervix Uteri Squamous Cell Carcinoma In Situ || Severe Squamous Dysplasia of the Cervix || Squamous Cell Carcinoma In Situ of Cervix || Squamous Cell Carcinoma In Situ of Cervix Uteri || Squamous Cell Carcinoma In Situ of Uterine Cervix || Squamous Cell Carcinoma In Situ of the Cervix || Squamous Cell Carcinoma In Situ of the Cervix Uteri || Squamous Cell Carcinoma In Situ of the Uterine Cervix || Uterine Cervix Squamous Cell Carcinoma In Situ Stage 0 includes: (Tis, N0, M0). Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. FIGO no longer includes stage 0. (AJCC 7th ed.) Malignant C80691 Chemotherapy-Related Leukemia A leukemia arising as a result of the mutagenic effect of chemotherapy agents that are used for the treatment of a malignant tumor. Malignant C44960 Chlamydia Psittaci-Associated Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Chlamydophila Psittaci-Associated Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Mucosa-associated lymphoid tissue lymphoma that arises from the ocular adnexa and caused by stimulation of lymphoid tissue due to Chlamydia psittaci bacterial infection. Malignant C4436 Cholangiocarcinoma A carcinoma that arises from the intrahepatic bile ducts, the hepatic ducts, or the common bile duct distal to the insertion of the cystic duct. The vast majority of tumors are adenocarcinomas. Malignant C4021 Chondroblastic Osteosarcoma Chondroblastic Osteogenic Sarcoma An osteosarcoma characterised by the presence of atypical cartilage of variable cellularity. It may or may not be associated with the presence of myxoid areas or focal bone formation. Malignant C2945 Chondroblastoma Chondroblastoma, NOS || Chondroblastoma, Not Otherwise Specified A benign, chondroid-producing, well-circumscribed, lytic neoplasm usually arising from the epiphysis of long bones. It is characterized by the presence of chondroblasts, osteoclast-like giant cells, chondroid formation, calcification, and mitotic activity. In aggressive cases, there is rearrangement of the 8q21 chromosome band. It occurs most frequently in children and young adults and rarely metastasizes. Benign C6902 Chondroid Chordoma A slow-growing malignant bone tumor arising from the remnants of the notochord and occurring in the base of the skull. The tumor is characterized by a lobulated growth pattern, myxoid stroma formation, and the presence of physaliphorous cells and cartilage. Malignant C53459 Chondroma A benign well circumscribed neoplasm of hyaline cartilage arising from bone or soft tissue. It is characterized by the presence of chondrocytes. Benign C2946 Chondrosarcoma A malignant cartilaginous matrix-producing mesenchymal neoplasm arising from the bone and soft tissue. It usually affects middle-aged to elderly adults. The pelvic bones, ribs, shoulder girdle, and long bones are the most common sites of involvement. Most chondrosarcomas arise de novo, but some may develop in a preexisting benign cartilaginous lesion. Malignant C6908 Chordoid Meningioma A WHO grade II, usually recurring meningioma characterized by the predominance of tissues that are histologically similar to chordoma. Undetermined C2947 Chordoma A malignant bone tumor arising from the remnants of the fetal notochord. Although it can occur at all ages, it is more frequently seen in middle-aged adults. The most frequent sites of involvement are the sacrococcygeal area, spheno-occipital area, and cervico-thoraco-lumbar spine. Chordomas tend to recur and may metastasize. The most common sites of metastasis are lung, bone, lymph nodes, and subcutaneous tissue. Malignant C2948 Choriocarcinoma Chorioepithelioma An aggressive malignant tumor arising from trophoblastic cells. The vast majority of cases arise in the uterus and represent gestational choriocarcinomas that derive from placental trophoblastic cells. Approximately half of the cases develop from a complete hydatidiform mole. A minority of cases arise in the testis or the ovaries. There is often marked elevation of human chorionic gonadotropin (hCG) in the blood. Choriocarcinomas disseminate rapidly through the hematogenous route; the lungs are most frequently affected. Malignant C4715 Choroid Plexus Carcinoma Cancer of Choroid Plexus || Cancer of the Choroid Plexus || Carcinoma of Choroid Plexus || Carcinoma of the Choroid Plexus || Choroid Plexus Cancer A malignant neoplasm arising from the choroid plexus. It shows anaplastic features and usually invades neighboring brain structures. Cerebrospinal fluid metastases are frequent. (Adapted from WHO) Malignant C4146 Chromophobe Renal Cell Carcinoma Chromophobe Adenocarcinoma || Chromophobe Carcinoma || Chromophobe Carcinoma of Kidney || Chromophobe Carcinoma of the Kidney || Chromophobe Cell Carcinoma of Kidney || Chromophobe Cell Carcinoma of the Kidney || Renal Cell Carcinoma, Chromophobe Type A type of carcinoma that comprises a minority of renal cell carcinomas. It is characterized by loss of chromosomes 1 and Y. Based on the cytoplasmic characteristics of the neoplastic cells, this type of carcinoma is classified as classic (typical) or eosinophilic. It has a much better prognosis than other renal cell carcinomas. Malignant C4563 Chronic Eosinophilic Leukemia, Not Otherwise Specified Chronic Eosinophilic Leukemia || Chronic Eosinophilic Leukemia, NOS || Eosinophilic Leukemia A rare myeloproliferative neoplasm characterized by a clonal proliferation of eosinophilic precursors resulting in persistently increased numbers of eosinophils in the blood, marrow and peripheral tissues. Since acute eosinophilic leukemia is at best exceedingly rare, the term eosinophilic leukemia is normally used as a synonym for chronic eosinophilic leukemia. In cases in which it is impossible to prove clonality and there is no increase in blast cells, the diagnosis of "idiopathic hypereosinophilic syndrome" is preferred. (WHO, 2001) Malignant C3163 Chronic Lymphocytic Leukemia B Cell CLL || B Cell Chronic Lymphocytic Leukemia || B Cell Lymphocytic Leukemia || B-Cell CLL || B-Cell Chronic Lymphocytic Leukemia || B-Cell Chronic Lymphoid Leukemia || B-Cell Lymphocytic Leukemia || Chronic B-Cell Lymphocytic Leukemia || Hematopoeitic - Chronic Lymphocytic Leukemia (CLL) The most common type of chronic lymphoid leukemia. It comprises 90% of chronic lymphoid leukemias in the United States. Morphologically, the neoplastic cells are small, round B-lymphocytes. This type of leukemia is not considered to be curable with available therapy. (WHO, 2001) Malignant C37202 Chronic Lymphocytic Leukemia with Immunoglobulin Heavy Chain Variable-Region Gene Somatic Hypermutation Postgerminal Center Chronic Lymphocytic Leukemia Chronic lymphocytic leukemia characterized by the presence of somatic hypermutation within the immunoglobulin heavy chain gene variable region of neoplastic clones. Patients have a better prognosis as compared to those with unmutated immunoglobulin heavy chain gene variable region rearrangements. Malignant C36272 Chronic Lymphocytic Leukemia with Plasmacytoid Differentiation A chronic lymphocytic leukemia characterized by the presence of neoplastic lymphocytes with plasmacytoid morphology. Malignant C37205 Chronic Lymphocytic Leukemia with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene Pregerminal Center Chronic Lymphocytic Leukemia Chronic lymphocytic leukemia characterized by the absence of somatic hypermutation within the immunoglobulin heavy chain gene variable region of neoplastic clones. Patients have a significantly worse prognosis as compared to those with mutated immunoglobulin heavy chain gene variable region rearrangements. Malignant C27911 Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma An indolent, mature B-cell neoplasm composed of small, round B-lymphocytes. When the bone marrow and peripheral blood are involved, the term chronic lymphocytic leukemia is used. The term small lymphocytic lymphoma is restricted to cases which do not show leukemic involvement of the bone marrow and peripheral blood. Malignant C37201 Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with Immunoglobulin Heavy Chain Variable-Region Gene Somatic Hypermutation Postgerminal Center Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) expressing somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes. The recognition of this variant alters the belief that CLL/SLL is always derived from a naive, pregerminal center B-cell. The presence of somatic hypermutations of IGH genes occurs in approximately 50% of CLL/SLL cases and implies a postgerminal center, memory origin. Patients with this variant of CLL/SLL have a favorable prognosis, with a reported median survival of more than 24 years. Malignant C37204 Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene Pregerminal Center Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) that lacks somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes, implying pregerminal center B-cell origin. Microarray gene expression profiling studies have demonstrated the expression of ZAP-70 gene (Syk family tyrosine kinase) in this subset of CLL/CLL. Patients with this variant of CLL/SLL have an unfavorable prognosis compared to those with somatic hypermutations of the IGH genes, with a median survival of approximately 6-8 years. Malignant C3174 Chronic Myelogenous Leukemia, BCR-ABL1 Positive BCR-ABL Positive Chronic Myelogenous Leukemia || CML - Chronic Myelogenous Leukemia || Chronic Granulocytic Leukemia || Chronic Myelocytic Leukemia || Chronic Myelogenous Leukemia || Chronic Myelogenous Leukemias || Chronic Myeloid Leukemia || Hematopoeitic - Chronic Myelocytic Leukemia (CML) A chronic myeloproliferative neoplasm characterized by the expression of the BCR-ABL1 fusion gene. It presents with neutrophilic leukocytosis. It can appear at any age, but it mostly affects middle aged and older individuals. Patients usually present with fatigue, weight loss, anemia, night sweats, and splenomegaly. If untreated, it follows a biphasic or triphasic natural course; an initial indolent chronic phase which is followed by an accelerated phase, a blast phase, or both. Allogeneic stem cell transplantation and tyrosine kinase inhibitors delay disease progression and prolong overall survival. Malignant C3178 Chronic Myelomonocytic Leukemia A myelodysplastic/myeloproliferative neoplasm which is characterized by persistent monocytosis, absence of a Philadelphia chromosome and BCR/ABL fusion gene, fewer than 20 percent blasts in the bone marrow and blood, myelodysplasia, and absence of PDGFRA or PDGFRB rearrangement. Malignant C36060 Chronic Myelomonocytic Leukemia with Eosinophilia CMML with Eosinophilia A chronic myelomonocytic leukemia characterized by a peripheral blood eosinophil count of equal or greater than 1.5x10E9/L, and absence of PDGFRA or PDGFRB gene abnormalities. Malignant C36061 Chronic Myelomonocytic Leukemia-1 A chronic myelomonocytic leukemia characterized by the presence of less than 10 percent blasts in the bone marrow and less than 5 percent blasts in the peripheral blood. Malignant C36062 Chronic Myelomonocytic Leukemia-2 A chronic myelomonocytic leukemia characterized by the presence of 10-19 percent blasts in the bone marrow and 5-19 percent blasts in the peripheral blood or by the presence of Auer rods regardless of the blasts count. Malignant C3179 Chronic Neutrophilic Leukemia Neutrophilic Leukemia A rare chronic myeloproliferative neoplasm characterized by neutrophilic leukocytosis. There is no detectable Philadelphia chromosome or BCR/ABL fusion gene. Malignant C3175 Chronic Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive Chronic Phase CML || Chronic Phase Chronic Granulocytic Leukemia || Chronic Phase Chronic Myelocytic Leukemia || Chronic Phase Chronic Myelogenous Leukemia || Chronic Phase Chronic Myeloid Leukemia A phase of chronic myelogenous leukemia in which the peripheral blood smear shows leukocytosis due mainly to neutrophils in different stages of maturation. Blasts usually account for less than 2% of the white blood cells counts. The platelet count is normal or increased. Thrombocytopenia is very uncommon during the chronic phase. Most patients have mild anemia. The bone marrow biopsy is hypercellular due to increased numbers of neutrophils and their precursors. Blasts usually account for fewer than 5% of the marrow cells, and more than 10% indicates transformation to the accelerated phase. Megakaryocytes are smaller than normal and have hypolobated nuclei. The spleen is enlarged due to infiltration of the cords of the red pulp by granulocytes. Most patients with chronic myelogenous leukemia are diagnosed in the chronic phase, which usually has an insidious onset and may last from several months to several years. (WHO, 2001) Malignant C66807 Ciliary Body Benign Medulloepithelioma Benign Ciliary Body Medulloepithelioma || Benign Intraocular Medulloepithelioma A rare, unilateral, benign embryonal neoplasm typically presenting as a ciliary body mass during childhood. It arises from primitive medullary epithelium. Benign C39814 Classic Congenital Mesoblastic Nephroma A congenital mesoblastic nephroma characterized by the presence of interlacing fascicles of fibroblastic cells, low mitotic activity, and collagen formation. Malignant C7164 Classic Hodgkin Lymphoma Classical Hodgkin Lymphoma || Classical Hodgkin's Lymphoma A monoclonal B-cell lymphoproliferation in the vast majority of cases. It is characterized by a bimodal age distribution (15-30 years of life and late life). Epstein-Barr virus has been postulated to play a role in the pathogenesis of classic Hodgkin lymphoma. Morphologically, it is characterized by the presence of Reed-Sternberg cells and mononuclear Hodgkin cells. The Reed-Sternberg and mononuclear Hodgkin cells are CD30 positive in nearly all cases and CD15 positive in the majority of cases. Four histologic subtypes have been distinguished: lymphocyte-rich, nodular sclerosis, mixed cellularity, and lymphocyte-depleted classic Hodgkin lymphoma. Malignant C54039 Classic Medulloblastoma A medulloblastoma composed of malignant cells with hyperchromatic nucleus and scanty cytoplasm. Homer Wright rosettes may be present. Malignant C7188 Classical Burkitt Lymphoma Classical Burkitt's Lymphoma A Burkitt lymphoma characterized by the presence of a uniform malignant lymphocytic infiltrate that is composed of medium-sized cells with round nuclei and multiple basophilic nucleoli, abundant mitotic figures, and a starry-sky pattern due to the presence of multiple tangible body macrophages. Malignant C111694 Classical Glioblastoma A molecular subtype of glioblastoma characterized by lack of p53 mutations, chromosome 7 amplifications or deletions, and high levels of EGFR amplification. Malignant C3766 Clear Cell Adenocarcinoma Clear Cell Carcinoma || Mesonephroid Clear Cell Adenocarcinoma || Mesonephroid Clear Cell Carcinoma A malignant neoplasm composed of glandular epithelial clear cells. Various architectural patterns may be seen, including papillary, tubulocystic, and solid. Malignant C6475 Clear Cell Chondrosarcoma A rare, usually low grade chondrosarcoma characterized by the presence of tumor cells with clear cytoplasm. It usually arises in the epiphyseal ends of long bones. Malignant C4714 Clear Cell Ependymoma An ependymoma, often supratentorial in location, characterized by the presence of ependymal cells with a perinuclear halo. Undetermined C4722 Clear Cell Meningioma A WHO grade II morphologic variant of meningioma characterized by the presence of clear glycogen-rich polygonal cells. Undetermined C4033 Clear Cell Renal Cell Carcinoma Clear Cell Carcinoma of Kidney || Clear Cell Carcinoma of the Kidney || Conventional (Clear Cell) Renal Cell Carcinoma || Conventional Renal Cell Carcinoma || Kidney Clear Cell Carcinoma || Renal Clear Cell Carcinoma A malignant epithelial neoplasm of the kidney characterized by the presence of lipid-containing clear cells within a vascular network. The tumor may metastasize to unusual sites and late metastasis is common. Malignant C9362 Clitoral Carcinoma Carcinoma of Clitoris || Carcinoma of the Clitoris || Clitoral Cancer || Clitoris Carcinoma A carcinoma that arises from the clitoris. Malignant C6194 Collecting Duct Carcinoma Carcinoma of Collecting Ducts of Bellini || Carcinoma of Kidney Collecting Duct || Carcinoma of Renal Collecting Duct || Carcinoma of the Kidney Collecting Duct || Carcinoma of the Renal Collecting Duct || Kidney Collecting Duct Carcinoma || Renal Collecting Duct Carcinoma Also known as collecting duct carcinoma, this is a rare type of renal carcinoma. It arises from the collecting ducts of the renal medulla, and most authors suggest that this is an aggressive tumor. Malignant C4349 Colon Adenocarcinoma Adenocarcinoma of Colon || Adenocarcinoma of the Colon || Colonic Adenocarcinoma An adenocarcinoma arising from the colon. It is more frequently seen in populations with a Western type diet and in patients with a history of chronic inflammatory bowel disease. Signs and symptoms include intestinal bleeding, anemia, and change in bowel habits. According to the degree of cellular differentiation, colonic adenocarcinomas are divided into well, moderately, and poorly differentiated. Histologic variants include mucinous adenocarcinoma, signet ring cell carcinoma, medullary carcinoma, serrated adenocarcinoma, cribriform comedo-type adenocarcinoma, and micropapillary adenocarcinoma. Malignant C3864 Colon Adenoma Adenoma of Colon || Adenoma of the Colon || Colonic Adenoma An adenoma that arises from the colon. The group of colonic adenomas includes tubular, villous, and tubulovillous adenomas, traditional serrated adenomas, sessile serrated adenomas/polyps, and familial adenomatous polyposis. Benign C128171 Colon Adenoma with Severe Dysplasia An adenoma that arises from the colon. It is characterized by the presence of severe epithelial dysplasia. Benign C96479 Colon Adenomatous Polyp Adenomatous Polyp of Colon || Adenomatous Polyp of the Colon || Colonic Adenomatous Polyp A polypoid adenoma that arises from and protrudes into the lumen of the colon. Epithelial dysplasia is always present. According to the architectural pattern it is classified as tubular, tubulovillous, or villous. Benign C5491 Colon Adenosquamous Carcinoma Adenosquamous Carcinoma of Colon || Adenosquamous Carcinoma of the Colon || Adenosquamous Colon Carcinoma || Colonic Adenosquamous Carcinoma An unusual colon carcinoma characterized by the presence of glandular and squamous carcinomatous components. The two carcinomatous components may be admixed within the tumor, or the two may appear separately in different areas. Malignant C4910 Colon Carcinoma Carcinoma of Colon || Carcinoma of the Colon || Colon Cancer || Colonic Carcinoma A malignant epithelial neoplasm that arises from the colon and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. Malignant C45429 Colon Intramucosal Adenocarcinoma Colon Intramucosal Neoplasia An adenocarcinoma that arises from the colonic glandular epithelium and invades the lamina propria or muscularis mucosa but not the submucosa. Malignant C60641 Colon Medullary Carcinoma A rare, invasive colon adenocarcinoma characterized by the presence of sheets of malignant epithelial cells with vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It usually has a favorable prognosis. Malignant C7966 Colon Mucinous Adenocarcinoma Colloid Adenocarcinoma of Colon || Colloid Adenocarcinoma of the Colon || Colloid Colon Adenocarcinoma || Colloidal Adenocarcinoma of Colon || Colloidal Adenocarcinoma of the Colon || Colloidal Colon Adenocarcinoma || Colon Colloid Adenocarcinoma || Colon Colloidal Adenocarcinoma || Colonic Colloid Adenocarcinoma || Colonic Colloidal Adenocarcinoma || Colonic Mucinous Adenocarcinoma || Mucinous Adenocarcinoma of Colon || Mucinous Adenocarcinoma of the Colon || Mucinous Colon Adenocarcinoma An invasive adenocarcinoma of the colon characterized by the presence of pools of extracellular mucin. Malignant glandular epithelial cells are present in the mucin collections. Mucin constitutes more than 50% of the lesion. Malignant C96486 Colon Serrated Adenocarcinoma A rare, invasive colon adenocarcinoma characterized by the presence of a malignant infiltrate with serrated glandular architecture. Malignant C96464 Colon Serrated Lesions and Polyps Colon SSA || Colon SSA/P || Colon SSP || Colon Sessile Serrated Adenoma || Colon Sessile Serrated Adenoma/Polyp || Colon Sessile Serrated Polyp Polyps that arises from the colon. They are characterized by the presence of serrated glands and the absence of generalized dysplasia. Benign C7967 Colon Signet Ring Cell Adenocarcinoma Colon Signet-Ring Cell Adenocarcinoma || Colonic Signet Ring Cell Adenocarcinoma || Signet Ring Cell Adenocarcinoma of Colon || Signet Ring Cell Adenocarcinoma of the Colon || Signet Ring Cell Colon Adenocarcinoma An invasive adenocarcinoma of the colon characterized by the presence of malignant glandular epithelial cells which contain prominent intracytoplasmic mucin (signet ring cells). The signet ring cells constitute more than 50% of the malignant cells. Malignant C6761 Colon Small Cell Neuroendocrine Carcinoma Colon Small Cell Carcinoma || Colonic Small Cell Carcinoma || Small Cell Carcinoma of Colon || Small Cell Carcinoma of the Colon || Small Cell Colon Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the colon. It is characterized by the presence of malignant small cells. Malignant C5490 Colon Squamous Cell Carcinoma Colon Epidermoid Carcinoma || Colonic Epidermoid Carcinoma || Colonic Squamous Cell Carcinoma || Epidermoid Carcinoma of Colon || Epidermoid Carcinoma of the Colon || Squamous Cell Carcinoma of Colon || Squamous Cell Carcinoma of the Colon || Squamous Cell Colon Carcinoma A very rare colon carcinoma characterized by the presence of a malignant squamous cell infiltrate. Malignant C43577 Colon Traditional Serrated Adenoma Colon Serrated Adenoma || Colon Serrated Adenoma Type II || Colon TSA An adenoma that arises from the colon. It is characterized by prominent serration of the glands. Benign C7041 Colon Tubular Adenoma A usually polypoid neoplasm that arises from the glandular epithelium of the colonic mucosa. It is characterized by a tubular architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C5496 Colon Tubulovillous Adenoma Colonic Tubulovillous Adenoma || Tubulovillous Adenoma of Colon || Tubulovillous Adenoma of the Colon A neoplasm that arises from the glandular epithelium of the colonic mucosa. It is characterized by tubular and villous architectural patterns. The neoplastic glandular cells have dysplastic features. Benign C7100 Colon Undifferentiated Carcinoma Carcinoma Simplex of the Colon. || Colon Anaplastic Carcinoma || Undifferentiated Carcinoma of Colon || Undifferentiated Carcinoma of the Colon || Undifferentiated Colonic Carcinoma An invasive malignant epithelial tumor that arises from the colon. There is no morphologic, immunophenotypic, or molecular biological evidence of glandular or squamous differentiation. Malignant C3495 Colon Villous Adenoma Colonic Villous Adenoma || Villous Adenoma of Colon || Villous Adenoma of the Colon A neoplasm that arises from the glandular epithelium of the colonic mucosa. It is characterized by a villous architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C5105 Colorectal Adenocarcinoma Adenocarcinoma of Large Bowel || Adenocarcinoma of Large Intestine || Adenocarcinoma of the Large Bowel || Adenocarcinoma of the Large Intestine || Large Bowel Adenocarcinoma || Large Intestine Adenocarcinoma The most common type of colorectal carcinoma. It is characterized by the presence of malignant glandular epithelial cells invading through the muscularis mucosa into the submucosa. Histologic variants include mucinous adenocarcinoma, signet ring cell carcinoma, medullary carcinoma, serrated adenocarcinoma, cribriform comedo-type adenocarcinoma, and micropapillary adenocarcinoma. Malignant C5673 Colorectal Adenoma Adenoma of Large Bowel || Adenoma of Large Intestine || Adenoma of the Large Bowel || Adenoma of the Large Intestine || Large Bowel Adenoma || Large Intestine Adenoma An adenoma that arises from the colon or rectum. The group of colorectal adenomas includes tubular, villous, and tubulovillous adenomas, traditional serrated adenomas, sessile serrated adenomas/polyps, and familial adenomatous polyposis. Benign C27458 Colorectal Adenoma with Mild Dysplasia An adenoma that arises from the colon or rectum. It is characterized by the presence of mild epithelial dysplasia. Benign C27457 Colorectal Adenoma with Moderate Dysplasia An adenoma that arises from the colon or rectum. It is characterized by the presence of moderate epithelial dysplasia. Benign C5685 Colorectal Adenoma with Severe Dysplasia Colorectal Adenoma with Marked Dysplasia An adenoma that arises from the colon or rectum. It is characterized by the presence of severe epithelial dysplasia. Benign C43589 Colorectal Adenosquamous Carcinoma An unusual colorectal carcinoma characterized by the presence of glandular and squamous carcinomatous components. The two carcinomatous components may be admixed within the tumor, or the two may appear separately in different areas. Malignant C2955 Colorectal Carcinoma Cancer of Large Bowel || Cancer of Large Intestine || Cancer of the Large Bowel || Cancer of the Large Intestine || Carcinoma of Large Bowel || Carcinoma of Large Intestine || Carcinoma of the Large Bowel || Carcinoma of the Large Intestine || Colorectal Cancer || Large Bowel Cancer || Large Bowel Carcinoma || Large Intestine Cancer || Large Intestine Carcinoma A malignant epithelial neoplasm that arises from the colon or rectum and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. Malignant C43590 Colorectal Medullary Carcinoma A rare, invasive colorectal adenocarcinoma characterized by the presence of sheets of malignant epithelial cells with vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It usually has a favorable prognosis. Malignant C43585 Colorectal Mucinous Adenocarcinoma An invasive colorectal adenocarcinoma characterized by the presence of extracellular mucin pools that contain malignant glandular epithelial structures. The extracellular mucin pools occupy more than 50% of the malignant lesion. Malignant C96485 Colorectal Serrated Adenocarcinoma A rare, invasive colorectal adenocarcinoma characterized by the presence of a malignant infiltrate with serrated glandular architecture. Malignant C83176 Colorectal Serrated Lesions and Polyps Colorectal Mixed Hyperplastic Adenomatous Polyp || Colorectal Mixed Hyperplastic Adenomatous Polyp/Serrated Adenoma || Colorectal SSA || Colorectal SSA/P || Colorectal SSP || Colorectal Sessile Serrated Adenoma || Colorectal Sessile Serrated Adenoma/Polyp || Colorectal Sessile Serrated Polyp Polyps that arises from the colon or rectum. They are characterized by the presence of serrated glands and the absence of generalized dysplasia. Benign C43586 Colorectal Signet Ring Cell Carcinoma Colorectal Signet-Ring Cell Carcinoma An invasive colorectal adenocarcinoma characterized by the presence of malignant glandular epithelial cells with prominent intracytoplasmic mucin resulting in the displacement of the nuclei. The malignant glandular cells with intracytoplasmic mucin constitute more than 50% of the malignant cellular infiltrate. Malignant C43587 Colorectal Small Cell Neuroendocrine Carcinoma Colorectal Small Cell Carcinoma || Large Intestinal Small Cell Neuroendocrine Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the colon or rectum. It is characterized by the presence of malignant small cells. Malignant C43588 Colorectal Squamous Cell Carcinoma A very rare colorectal carcinoma characterized by the presence of a malignant squamous cell infiltrate. Malignant C5674 Colorectal Traditional Serrated Adenoma Colorectal Serrated Adenoma || Colorectal Serrated Adenoma Type II || Colorectal TSA || Large Bowel Traditional Serrated Adenoma || Traditional Serrated Adenoma of Large Bowel || Traditional Serrated Adenoma of the Large Bowel An adenoma that arises from the colon or rectum. It is characterized by prominent serration of the glands. Benign C27456 Colorectal Tubular Adenoma A usually polypoid neoplasm that arises from the glandular epithelium of the colonic and rectal mucosa. It is characterized by a tubular architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C5675 Colorectal Tubulovillous Adenoma Large Bowel Tubulovillous Adenoma || Tubulovillous Adenoma of Large Bowel || Tubulovillous Adenoma of the Large Bowel A neoplasm that arises from the glandular epithelium of the colonic and rectal mucosa. It is characterized by tubular and villous architectural patterns. The neoplastic glandular cells have dysplastic features. Benign C43591 Colorectal Undifferentiated Carcinoma An invasive malignant epithelial tumor that arises from the colon or rectum. There is no morphologic, immunophenotypic, or molecular biological evidence of glandular or squamous differentiation. Malignant C5676 Colorectal Villous Adenoma Large Bowel Villous Adenoma || Villous Adenoma of Large Bowel || Villous Adenoma of the Large Bowel A neoplasm that arises from the glandular epithelium of the colonic and rectal mucosa. It is characterized by a villous architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C3828 Combined Hepatocellular Carcinoma and Cholangiocarcinoma Carcinoma of Liver and Intrahepatic Biliary Tract || Carcinoma of the Liver and Intrahepatic Biliary Tract || Cholangiohepatoma || Hepatocholangiocarcinoma || Liver and Intrahepatic Biliary Tract Carcinoma || Mixed Hepatocellular Cholangiocarcinoma A rare tumor containing unequivocal elements of both hepatocellular and cholangiocarcinoma that are intimately admixed. This tumor should be distinguished from separate hepatocellular carcinoma and cholangiocarcinoma arising in the same liver. The prognosis of this tumor is poor. Malignant C45475 Composite Hemangioendothelioma An intermediate, locally aggressive and rarely metastasizing blood vessel neoplasm. It is characterized by the presence of spindle, plump, and epithelioid endothelial cells and cellular atypia. Undetermined C38661 Composite Lymphoma Coexistence of Hodgkin and non-Hodgkin lymphoma in the same anatomic site. Malignant C3901 Compound Nevus Compound Nevus of Skin || Compound Nevus of the Skin A nevus composed of neoplastic melanocytes that infiltrate both the epidermis and the dermis. Benign C6569 Congenital Mesoblastic Nephroma Mesoblastic Nephroma A low grade childhood congenital malignant neoplasm arising from the kidney. It is characterized by the presence of fibroblastic cells. The majority of cases occur in the first year of life. Complete excision is usually associated with an excellent prognosis. Malignant C3841 Congenital Non-Progressive Hemangioma Congenital Angioma || Congenital Hemangioma A hemangioma present at birth. Benign C4549 Conjunctival Squamous Cell Carcinoma Conjunctiva Epidermoid Carcinoma || Conjunctiva Squamous Cell Carcinoma || Conjunctival Epidermoid Carcinoma || Epidermoid Carcinoma of Conjunctiva || Epidermoid Carcinoma of the Conjunctiva || Squamous Cell Carcinoma of Conjunctiva || Squamous Cell Carcinoma of the Conjunctiva A low-grade squamous cell carcinoma that arises from the conjunctiva. It is the most common primary malignant tumor that arises from the conjunctiva. It usually affects older white males. Excessive exposure to sunlight is a risk factor. Patients may present with a mass, red eye, or pain. Malignant C9428 Conventional Leiomyosarcoma Classic Leiomyosarcoma An uncommon, aggressive malignant smooth muscle neoplasm. It is characterized by the presence of atypical large spindle or round cells, nuclear palisading, tumor cell necrosis, mitotic figures and may be associated with vascular invasion. Malignant C35870 Conventional Osteosarcoma Central Osteosarcoma || Conventional Central Osteosarcoma || Intracortical Osteogenic Sarcoma || Intracortical Osteosarcoma || Medullary Osteosarcoma A high grade malignant bone-forming mesenchymal neoplasm producing osteoid. The tumor arises from the medullary portion of the bone. It affects the long bones and most commonly, the distal femur, proximal tibia, and proximal humerus. Pain with or without a palpable mass is the most common clinical presentation. It usually has an aggressive growth and may metastasize through the hematogenous route. The lung is the most frequent site of metastasis. Malignant C122688 Core Binding Factor Acute Myeloid Leukemia Acute myeloid leukemia characterized by the presence of t(8;21)(q22;q22) or inv(16)(p13q22)/t(16;16)(p13;q22). These cytogenetic abnormalities result in disruption of the transcription factor CBF, which is a regulator of normal hematopoiesis. Malignant C4552 Corneal Squamous Cell Carcinoma Cornea Epidermoid Carcinoma || Cornea Squamous Cell Carcinoma || Corneal Epidermoid Carcinoma || Epidermoid Carcinoma of Cornea || Epidermoid Carcinoma of the Cornea || Squamous Cell Carcinoma of Cornea || Squamous Cell Carcinoma of the Cornea A rare squamous cell carcinoma that arises from the cornea. Malignant C7462 Corticotroph Adenoma ACTH Producing Pituitary Gland Adenoma || ACTH Secreting Adenoma of Pituitary || ACTH Secreting Adenoma of the Pituitary || ACTH-Producing Pituitary Gland Adenoma || ACTH-Secreting Adenoma of Pituitary || ACTH-Secreting Adenoma of Pituitary Gland || ACTH-Secreting Adenoma of the Pituitary || ACTH-Secreting Adenoma of the Pituitary Gland || ACTHoma || Adrenocorticotropin Producing Adenoma of Pituitary || Adrenocorticotropin Producing Adenoma of Pituitary Gland || Adrenocorticotropin Producing Adenoma of the Pituitary || Adrenocorticotropin Producing Adenoma of the Pituitary Gland || Adrenocorticotropin Producing Pituitary Adenoma || Adrenocorticotropin Producing Pituitary Gland Adenoma || Adrenocorticotropin Secreting Adenoma of Pituitary || Adrenocorticotropin Secreting Adenoma of Pituitary Gland || Adrenocorticotropin Secreting Adenoma of the Pituitary || Adrenocorticotropin Secreting Adenoma of the Pituitary Gland || Adrenocorticotropin Secreting Pituitary Adenoma || Adrenocorticotropin Secreting Pituitary Gland Adenoma || Corticotropic Adenoma || Corticotropin Secreting Adenoma of the Pituitary || Corticotropin Secreting Pituitary Gland Adenoma || Corticotropinoma || Pituitary ACTH Secreting Adenoma || Pituitary ACTH-Secreting Adenoma || Pituitary Corticotropin Secreting Adenoma || Pituitary Gland ACTH-Secreting Adenoma An adenoma of the pituitary gland that produces corticotropin. The vast majority of cases are associated with Cushing disease. Clinical manifestations include truncal obesity with thin extremities, thinning of the skin, osteoporosis, and a tendency to bruise easily. Silent or hormonally non-functioning ACTH producing adenomas have also been described. They produce symptoms of a mass-related lesion. Benign C121963 Cribriform Neuroepithelial Tumor A very rare, nonrhabdoid, intraventricular tumor with relatively favorable prognosis. It is characterized by the presence of neuroepithelial cells forming cribriform patterns, trabeculae, epithelial membrane antigen immunopositivity on epithelial surfaces, and loss of nuclear INI 1 expression. Undetermined C3510 Cutaneous Melanoma Malignant Cutaneous Melanoma || Malignant Melanoma (of Skin), Stage Unspecified || Malignant Melanoma of Skin || Malignant Melanoma of Skin Stage Unspecified || Melanoma of Skin || Melanoma of the Skin || Skin Melanoma || Skin, Melanoma A primary melanoma arising from atypical melanocytes in the skin. Precursor lesions include acquired and congenital melanocytic nevi, and dysplastic nevi. Several histologic variants have been recognized, including superficial spreading melanoma, acral lentiginous melanoma, nodular melanoma, and lentigo maligna melanoma. Malignant C4225 Cutaneous Nodular Melanoma Nodular Malignant Melanoma of Skin || Nodular Malignant Melanoma of the Skin || Nodular Malignant Skin Melanoma An aggressive form of melanoma, frequently metastasizing to the lymph nodes. It presents as a papular or nodular raised skin lesion. It comprises approximately 10-15% of melanomas. Morphologically, it often displays an epithelioid appearance. Malignant C27094 Cylindroma Cylindroma of Skin || Cylindroma of the Skin || Dermal Cylindroma A benign sweat gland neoplasm usually occurring in the scalp or the face. It may present as solitary or multiple papular or nodular lesions. It may be a sporadic lesion or part of Brooke-Spiegler syndrome. It arises from the dermis and has a multinodular, circumscribed appearance. The nodules contain basaloid cells with small, dark nuclei. Complete excision is usually curative. Benign C2971 Cystadenocarcinoma A malignant cystic epithelial neoplasm arising from the glandular epithelium. The malignant epithelial cells invade the stroma. The cystic spaces contain serous or mucinous fluid. Representative examples include ovarian and pancreatic cystadenocarcinomas. Malignant C122687 Cytogenetically Normal Acute Myeloid Leukemia Normal Karyotype Acute Myeloid Leukemia Acute myeloid leukemia not associated with cytogenetic abnormalities. Malignant C121975 DDIT4L Acute Lymphoblastic Leukemia DDIT4L ALL A gene expression subtype of acute lymphoblastic leukemia characterized by its expression level of the DDIT4L gene. Malignant C6476 Dedifferentiated Chondrosarcoma An aggressive morphologic variant of chondrosarcoma. It is composed of a low grade chondrosarcoma and a high grade non-cartilagenous sarcomatous component. Due to the aggressive nature of the disease, its prognosis is poor. Malignant C48876 Dedifferentiated Chordoma Sarcomatoid Chordoma A high-grade malignant bone tumor arising from the remnants of the notochord. It is characterized by a lobulated growth pattern, myxoid stroma formation, the presence of physaliphorous cells, and a sarcomatous component. Malignant C3704 Dedifferentiated Liposarcoma An atypical lipomatous tumor/well differentiated liposarcoma that shows progression to a usually non-lipomatous, high grade sarcoma. The non-lipomatous sarcoma component may be present in the primary lesion or at the site of recurrence. Malignant C6492 Deep Fibrous Histiocytoma Benign Deep Fibrous Histiocytoma || Deep Benign Fibrous Histiocytoma A rare, well-circumscribed, pseudo-encapsulated benign fibrous histiocytoma that arises entirely within the subcutaneous tissue or deep soft tissue. It usually affects the extremities or the head and neck region. It recurs locally in a minority of cases. Benign C6555 Deep Hemangioma Deep Angioma A hemangioma arising from the deep soft tissues. Benign C6484 Dermal Fibroma Fibroma of Skin || Fibroma of the Skin || Skin Fibroma A benign neoplasm arising from the dermis. It is characterized by the presence of spindle-shaped fibroblasts. Benign C3804 Dermal Nevus Intradermal Nevus A nevus characterized by the proliferation of nevus cells in the dermis without involvement of the dermal-epidermal junction. Benign C4683 Dermatofibrosarcoma Protuberans Dermatofibrosarcoma A low grade fibroblastic neoplasm presenting as a nodular cutaneous mass, most often on the trunk and the proximal extremities. The tumor diffusely infiltrates the dermis and the subcutaneous tissues. It is considered a locally aggressive neoplasm, which often recurs but rarely metastasizes. Undetermined C9182 Desmoid Fibromatosis Aggressive Fibromatosis || Deep Fibromatosis || Deep Fibromatosis/Desmoid Tumor || Desmoid Tumor || Desmoid-Type Fibromatosis An insidious, locally aggressive, poorly circumscribed neoplasm arising from the deep soft tissues. It is characterized by the presence of elongated spindle-shaped fibroblasts, collagenous stroma formation, and an infiltrative growth pattern. It lacks metastatic potential. Undetermined C9476 Desmoplastic Infantile Astrocytoma Desmoplastic Astrocytoma of Infancy A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO) Undetermined C4738 Desmoplastic Infantile Ganglioglioma A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population of neoplastic astrocytes together with a variable neuronal component. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as a large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO) Undetermined C6747 Desmoplastic Mesothelioma A malignant neoplasm arising from mesothelial cells. It is characterized by the presence of a dense collagenous tissue and atypical neoplastic cells. Sarcomatoid features, collagenous necrosis, and infiltration of muscle and adipose tissue may be present. It occurs in the pleura and less commonly in the peritoneum. Malignant C4497 Desmoplastic Nevus A benign melanocytic nevus characterized by the presence of desmoplastic stroma. Benign C4956 Desmoplastic/Nodular Medulloblastoma Desmoplastic Medulloblastoma || Desmoplastic Nodular Medulloblastoma A medulloblastoma characterized by the presence of nodular, collagenous areas which do not contain reticulin, surrounded by hypercellular areas which contain an intercellular reticulin fiber network. Malignant C66813 Differentiated Retinoblastoma A retinoblastoma with well differentiated features. It often produces Flexner-Wintersteiner rosettes or Homer-Wright rosettes. In some cases the tumor cells form fleurettes. Malignant C42048 Differentiating Neuroblastoma A neuroblastoma in which the differentiating neuroblasts constitute more than five-percent of the tumor cells. Malignant C7173 Diffuse Astrocytoma Grade II Astrocytic Neoplasm || Grade II Astrocytic Tumor || Grade II Astrocytoma || WHO Grade II Astrocytoma A low-grade (WHO grade II) astrocytic neoplasm. It is characterized by diffuse infiltration of neighboring central nervous system structures. These lesions typically affect young adults and have a tendency for progression to anaplastic astrocytoma and glioblastoma. Based on the IDH genes mutation status, diffuse astrocytomas are classified as IDH-mutant, IDH-wildtype, and not otherwise specified. Undetermined C129271 Diffuse Astrocytoma, IDH-Mutant Diffuse astrocytoma carrying IDH mutations. Undetermined C129277 Diffuse Astrocytoma, Not Otherwise Specified Diffuse Astrocytoma, NOS Diffuse astrocytoma in which there is insufficient information on the IDH genes status. Undetermined C37209 Diffuse Blastoid B-Cell Lymphoma Blastoid Follicular Lymphoma A distinct subtype of high-grade follicular center cell lymphoma, representing a diffuse transformation of conventional follicular lymphoma. Although this type of lymphoma strongly expresses BCL-2, it does not carry the characteristic t(14;18)(q32;q21) chromosomal translocation found in follicular lymphomas. Instead, extra copies of chromosome 18 are often detected. Malignant C7264 Diffuse Follicular Lymphoma Diffuse Follicle Centre Lymphoma || Follicle Centre Lymphoma Diffuse A lymphoma with the morphologic and immunophenotypic features of a follicular lymphoma that shows entirely a diffuse pattern. It is usually seen in small biopsy specimens and most probably represents a diffuse area of a follicular lymphoma. Malignant C129325 Diffuse Glioma A glioma that has diffusely infiltrated the surrounding central nervous system tissues. Undetermined C8851 Diffuse Large B-Cell Lymphoma A non-Hodgkin lymphoma characterized by a diffuse proliferation of predominantly large neoplastic B lymphocytes. It is the most frequently seen type of non-Hodgkin lymphoma, representing 30%-40% of the cases. Morphologic variants include centroblastic lymphoma, immunoblastic lymphoma, and anaplastic lymphoma. Subtypes/entities include T-cell/histiocyte rich large B-cell lymphoma, primary diffuse large B-cell lymphoma of the central nervous system, plasmablastic lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg type, and ALK-positive large B-cell lymphoma. Malignant C36081 Diffuse Large B-Cell Lymphoma Activated B-Cell Type Activated B-Cell-Like Diffuse Large B-Cell Lymphoma || DLBCL Activated B-Cell Type || Diffuse Large B-Cell Lymphoma Non-GC/ABC || Diffuse Large B-Cell Lymphoma Non-Germinal Center/Activated B-Cell Type || Diffuse Large B-Cell Lymphoma with an Activated B-Cell Expression Profile || Non-GC/ABC DLBCL A biologic subset of diffuse large B-cell lymphomas with a unique molecular signature or expression profile. It represents approximately 30% of diffuse large B-cell lymphomas, and is characterized by the expression of CD44, PKCbeta1, Cyclin D2, BCL-2, and IRF4/MUM1 genes. Morphologically, these lymphomas are either centroblastic or immunoblastic (ratio 2:1). Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome compared to those with a germinal center B-cell expression profile, with a 5-year survival rate of 35% and a median survival of 2 years. Malignant C80289 Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation A diffuse large B-cell lymphoma arising in body cavities or narrow spaces of long standing chronic inflammation. The classic example is the pyothorax-associated lymphoma that arises in the pleural cavity of patients with a history of long standing pyothorax. Malignant C36080 Diffuse Large B-Cell Lymphoma Germinal Center B-Cell Type DLBCL Germinal Center B-Cell Type || Diffuse Large B-Cell Lymphoma with a Germinal Center B-Cell Expression Profile || Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma A biologic subset of diffuse large B-cell lymphomas with a unique molecular signature or expression profile. It represents approximately 50% of diffuse large B-cell lymphomas, and is characterized by the expression of CD10, BCL-6, A-myb, and LMO2 genes, BCL-2 translocation, and c-REL amplification. Morphologically, the vast majority of these lymphomas are centroblastic and a small minority are immunoblastic (ratio20:1). Patients with this type of diffuse large B-cell lymphoma have a more favorable outcome, with a 5-year survival rate of 60% and a median survival of 10 years. Malignant C45231 Diffuse Large B-Cell Lymphoma Unclassifiable DLBCL Unclassifiable || Diffuse Large B-Cell Lymphoma Indeterminate || Type 3 Diffuse Large B-Cell Lymphoma A biologic subset of diffuse large B-cell lymphomas (DLBCL) that do not overexpress the genes whose overexpression characterizes the germinal center B-cell-like and activated B-cell-like DLBCL. Morphologically, these lymphomas are either immunoblastic or centroblastic. Patients with this type of diffuse large B-cell lymphoma are reported to have a less favorable outcome, similar to those with an activated B-cell-like gene expression profile. Malignant C80280 Diffuse Large B-Cell Lymphoma, Not Otherwise Specified A term referring to a group of diffuse large B-cell lymphomas which are biologically heterogeneous. These lymphomas have a centroblastic, immunoblastic, or anaplastic morphology. Malignant C45219 Diffuse Large B-Cell Lymphoma, Not Otherwise Specified by Gene Expression Profile A header term that includes the following diffuse large B-cell lymphoma subtypes by gene expression profiling: activated B-cell-like diffuse large B-cell lymphoma, germinal center B-cell-like diffuse large B-cell lymphoma, and type 3 diffuse large B-cell lymphoma. Malignant C8420 Diffuse Malignant Mesothelioma Malignant Diffuse Mesothelioma A malignant neoplasm that arises from the mesothelial cells. Histologic variants include biphasic, epithelioid, sarcomatoid, and desmoplastic mesothelioma. Malignant C66815 Diffuse Retinoblastoma A retinoblastoma characterized by the absence of a distinct retinal mass and the presence of malignant cells diffusely infiltrating the retina. It is often confused with uveitis and endophthalmitis, resulting in delayed diagnosis of the malignancy. Malignant C36207 Digestive System Adenoma GI System Adenoma || Gastrointestinal System Adenoma A sporadic or less frequently familial neoplasm that arises from the glandular epithelium of the gastrointestinal tract and liver. In the gastrointestinal tract, it manifests as a polypoid or flat circumscribed lesion. Morphologically, it is characterized by a proliferation of neoplastic glandular cells and is associated with dysplasia. Based on the growth pattern, it may be classified as tubular, villous, or tubulovillous. The dysplasia is classified as mild, moderate, or severe. The frequency of malignant transformation depends on the size of the lesion and the degree of dysplasia. Larger gastrointestinal tract adenomas with severe dysplastic changes carry a higher risk of progression to invasive adenocarcinoma. Gastrointestinal adenomas may present as solitary or multifocal lesions. In the liver, it arises from the hepatocytes. Grossly, it appears as a soft, round mass. Morphologically, the neoplastic cells resemble normal hepatocytes. Benign C7709 Digestive System Neuroendocrine Tumor G1 Carcinoid Tumor of Digestive System || Carcinoid Tumor of GI System || Carcinoid Tumor of Gastrointestinal System || Carcinoid Tumor of the Digestive System || Carcinoid Tumor of the GI System || Carcinoid Tumor of the Gastrointestinal System || Digestive Carcinoid Tumor || Digestive System Carcinoid Tumor || GI Carcinoid Tumor || Gastrointestinal Carcinoid Tumor || Gastrointestinal NET G1 || Gastrointestinal Neuroendocrine Tumor G1 || Gastrointestinal System Carcinoid Tumor A usually small, slow-growing, well differentiated neuroendocrine tumor arising from the digestive system. It is composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with small vesicular nuclei. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent. The tumor can occur anywhere in the digestive system; approximately 90% arise in the appendix. Undetermined C96166 Digestive System Neuroendocrine Tumor G2 Gastrointestinal NET G2 || Gastrointestinal Neuroendocrine Tumor G2 A well differentiated, intermediate grade tumor with neuroendocrine differentiation that arises from the digestive system. The mitotic count is 2-20 per 10 HPF and/or the Ki67 index is 3 to 20 percent. Undetermined C81772 Disseminated Juvenile Xanthogranuloma Disseminated JXG Juvenile xanthogranuloma disseminated to extracutaneous sites including mucosal surfaces, lung, central nervous system, pituitary, lymph nodes, eye, liver, and bone marrow. It is characterized by the presence of lipid-laden, foamy histiocytes and Touton-type giant cells forming nodules in the affected anatomic sites. Undetermined C7109 Distal Bile Duct Carcinoma A carcinoma that arises from the common bile duct distal to the insertion of the cystic duct. Malignant C115210 Distal Urethral Carcinoma Distal Urethra Carcinoma A carcinoma that arises from the distal part of the urethra. Malignant C7889 Duodenal Adenocarcinoma Adenocarcinoma of Duodenum || Adenocarcinoma of the Duodenum An adenocarcinoma that arises from the duodenum. Histologic variants include mucinous adenocarcinoma and signet ring cell carcinoma. Malignant C9505 Dysembryoplastic Neuroepithelial Tumor Dysembryoplastic Neuroepithelial Neoplasm A benign glial-neuronal neoplasm. It is usually supratentorial, located in the cortex. It occurs in children and young adults with a long-standing history of partial seizures. A histologic hallmark of this tumor is the 'specific glioneuronal element', characterized by columns, made up of bundles of axons, oriented perpendicularly to the cortical surface. (Adapted from WHO) Undetermined C8419 Dysplastic Cerebellar Gangliocytoma Dysplastic Gangliocytoma of Cerebellum || Dysplastic Gangliocytoma of the Cerebellum || Lhermitte-Duclos Disease A benign, WHO grade I cerebellar mass, which occurs in young adults and is composed of dysplastic ganglion cells. It is the major CNS manifestation of Cowden disease, an autosomal dominant condition that causes a variety of hamartomas and neoplasms. Undetermined C3694 Dysplastic Nevus Atypical Nevus || Clark Nevus || Clark's Nevus || Lentiginous Nevus || Nevus with Architectural Disorder || Nevus with Architectural Disorder and Cytologic Atypia of Melanocytes Solitary or multiple, slightly raised pigmented melanocytic lesions with irregular borders, usually measuring more than 0.6cm in greatest dimension. Morphologically, there is melanocytic atypia and the differential diagnosis from melanoma may be difficult. Patients are at an increased risk for the development of melanoma. Undetermined C54262 Ear Carcinoma A carcinoma that arises from the ear. Representative examples include ceruminous adenocarcinoma and squamous cell carcinoma of the external ear and adenocarcinoma of the middle ear. Malignant C27131 Early Gastric Cancer Microinvasive Gastric Cancer || Superficial Gastric Cancer || Superficial Spreading Gastric Cancer || Surface Gastric Cancer An invasive adenocarcinoma confined to the mucosa or mucosa and submucosa of the gastric wall. The regional lymph nodes may or may not be involved. It usually occurs in the lesser curvature. The 5-year survival rate following resection is between 80 percent and 95 percent, and remains high even when lymph node metastases are present. Malignant C96772 Early Hepatocellular Carcinoma A low grade, well differentiated small hepatocellular carcinoma. It lacks obvious stromal invasion and significant atypia. Malignant C80281 EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified EBV-Positive DLBCL, NOS || Epstein-Barr Virus Positive DLBCL, NOS || Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified A diffuse large B-cell lymphoma originally described in patients older than 50 years but it has been increasingly recognized in younger patients. Epstein-Barr virus is present in all cases. There is no known history of immunodeficiency or prior lymphoma. Malignant C27695 EBV-Related Clonal Post-Transplant Lymphoproliferative Disorder EBV Related Clonal PTLD || EBV-Related Clonal PTLD || Epstein-Barr Virus-Related Clonal Post-Transplant Lymphoproliferative Disorder A clonal lymphoproliferative disorder that develops following an organ transplantation and is caused by Epstein-Barr virus infection. Malignant C27255 Eccrine Carcinoma An adenocarcinoma with eccrine differentiation arising from the sweat glands. It includes the following subtypes: ductal eccrine adenocarcinoma, papillary eccrine carcinoma, and eccrine porocarcinoma. Malignant C7565 Eccrine Hidrocystoma Eccrine Cystadenoma A benign sweat gland cystic lesion that arises from the dermis. It is lined by a thin epithelial layer of cells with a slightly eosinophilic cytoplasm. Benign C3752 Embryonal Carcinoma A non-seminomatous malignant germ cell tumor characterized by the presence of large germ cells with abundant cytoplasm resembling epithelial cells, geographic necrosis, high mitotic activity, and pseudoglandular and pseudopapillary structures formation. It can arise from the testis, ovary, and extragonadal sites (central nervous system and mediastinum). Malignant C8971 Embryonal Rhabdomyosarcoma A poorly circumscribed morphologic variant of rhabdomyosarcoma. It is characterized by the presence of primitive skeletal muscle differentiation in any stage of myogenesis. Malignant C4915 Embryonal Tumor with Multilayered Rosettes, C19MC-Altered ETMR, C19MC-Altered || Embryonal Tumor with Abundant Neuropil and True Rosettes || Embryonal Tumor with Multilayered Rosettes || Embryonal Tumor with Multilayered Rosettes with C19MC Amplification || Ependymoblastoma An aggressive malignant embryonal neoplasm arising from the central nervous system. It is characterized by the presence of multilayered rosettes formation and the presence of amplification of the C19MC region on chromosome 19 (19q13.42). Malignant C7386 Encapsulated Thymoma A thymoma that is confined within the capsule and may display benign or malignant morphologic characteristics. Undetermined C3007 Enchondroma Central Chondroma A common benign hyaline cartilage neoplasm arising in the intramedullary bone. It is characterized by the presence of chondrocytes, low mitotic activity, and in some cases, a nodular pattern and calcification. The small bones of the hands and feet are the most frequently affected sites. Benign C34584 Endocervical Polyp A polyp that arises from the endocervix. It is characterized by the presence of endocervical glands and a fibrovascular stroma. Benign C7359 Endometrial Adenocarcinoma Adenocarcinoma of Endometrium || Adenocarcinoma of the Endometrium || Endometrial Adenocarcinoma, Not Otherwise Specified An adenocarcinoma arising from the uterine body cavity. This is the most frequent malignant tumor affecting the uterine body, and is linked to estrogen therapy. Most patients present with uterine bleeding and are over age 40 at the time of diagnosis. The prognosis depends on the stage of the tumor, the depth of the uterine wall invasion, and the histologic subtype. Endometrioid adenocarcinoma is the most frequently seen morphologic variant of endometrial adenocarcinoma. Malignant C114656 Endometrial Adenosquamous Carcinoma A rare endometrial carcinoma characterized by the presence of both malignant glandular and malignant squamous cellular components. Malignant C7558 Endometrial Carcinoma Carcinoma of Endometrium || Carcinoma of the Endometrium A malignant tumor arising from the epithelium that lines the cavity of the uterine body. The vast majority of endometrial carcinomas are adenocarcinomas; squamous cell and adenosquamous carcinomas represent a minority of the cases. Endometrioid adenocarcinoma is the most frequently seen variant of endometrial adenocarcinoma. Uterine bleeding is an initial clinical sign. The prognosis depends on the stage of the tumor, the depth of myometrial wall invasion, and the degree of differentiation. Malignant C8028 Endometrial Clear Cell Adenocarcinoma Clear Cell Carcinoma of Endometrium || Clear Cell Carcinoma of the Endometrium A high-grade endometrial adenocarcinoma characterized by the presence of large neoplastic cells that display at least focal cytoplasmic clearing. The neoplastic cells form papillary, glandular, or sheet-like structures. Malignant C6287 Endometrial Endometrioid Adenocarcinoma Endometrial Endometrioid Carcinoma || Endometrioid Adenocarcinoma of Endometrium || Endometrioid Adenocarcinoma of the Endometrium || Endometrioid Carcinoma of Endometrium || Endometrioid Carcinoma of the Endometrium || Uterine Corpus Endometrioid Adenocarcinoma || Uterine Corpus Endometrioid Carcinoma A primary endometrial adenocarcinoma composed of neoplastic cells that form complex glandular patterns associated with budding and branching of the neoplastic glands. The neoplastic glands resemble those of the normal endometrium and may or may not be associated with sheet-like proliferation of malignant cells. Endometrioid adenocarcinoma is the most commonly seen morphologic variant of endometrial adenocarcinoma. Malignant C40144 Endometrial Mucinous Adenocarcinoma Uterine Corpus Mucinous Adenocarcinoma A primary, usually low grade adenocarcinoma of the endometrium in which the majority of the malignant epithelial cells contain abundant intracytoplasmic mucin. Malignant C27838 Endometrial Serous Adenocarcinoma Uterine Corpus Serous Adenocarcinoma || Uterine Papillary Serous Carcinoma || Uterine Serous Adenocarcinoma || Uterine Serous Carcinoma || Uterine Serous Papillary Adenocarcinoma A high grade, aggressive adenocarcinoma arising from the endometrium. It is characterized by the presence of complex papillary patterns with cellular budding. Atypical mitoses, necrosis, and psammoma bodies may be present. It is classified as type II endometrial carcinoma and it is not associated with endometrial hyperplasia. It tends to invade deeply into the myometrium and spreads into the lymphatic vessels. Patients frequently present with spread of the tumor beyond the uterus at the time of diagnosis. The prognosis is usually poor. Malignant C40155 Endometrial Small Cell Neuroendocrine Carcinoma Endometrial Small Cell Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the endometrium. It is characterized by the presence of small malignant cells, necrotic changes, and an increased mitotic rate. Malignant C8719 Endometrial Squamous Cell Carcinoma Squamous Cell Carcinoma of Endometrium || Squamous Cell Carcinoma of the Endometrium A primary carcinoma of the endometrium characterized by the presence of malignant squamous cells. Malignant C8973 Endometrioid Stromal Sarcoma Endometrial Stromal Sarcoma A malignant mesenchymal neoplasm that affects the uterine corpus, and rarely, the ovaries, cervix, and vagina. In the uterine corpus it is classified as low grade or high grade endometrial stromal sarcoma. In the remainder of the anatomic sites it is classified as low grade endometrioid stromal sarcoma. Malignant C4737 Enteropathy-Associated T-Cell Lymphoma EATL, Type I || Enteropathy Associated T-Cell Lymphoma || Enteropathy-Associated T-Cell Lymphoma, Type I || Enteropathy-Type T-Cell Lymphoma A mature T-cell and NK-cell non-Hodgkin lymphoma of intraepithelial T-lymphocytes. It usually arises from the small intestine, most commonly the jejunum or ileum. Other less frequent primary anatomic sites include the duodenum, stomach, colon, or outside the gastrointestinal tract. It is characterized by the presence of pleomorphic medium-sized to large T-lymphocytes with vesicular nuclei, prominent nucleoli, and moderate to abundant pale cytoplasm. It is associated with celiac disease. Malignant C3016 Eosinophilic Granuloma Eosinophilic Xanthomatous Granuloma A clinical variant of Langerhans cell histiocytosis characterized by unifocal involvement of a bone (most often), skin, or lung. Patients are usually older children or adults usually presenting with a lytic bone lesion. The etiology is unknown. Morphologically, eosinophilic granuloma is characterized by the presence of Langerhans cells in a characteristic milieu which includes histiocytes, eosinophils, neutrophils, and small, mature lymphocytes. Undetermined C6770 Ependymal Tumor Ependymal Neoplasm A group of neoplasms which arise from the ependymal lining of the cerebral ventricles and from the remnants of the central canal of the spinal cord. Ependymal tumors occur predominantly in children and young adults with varied morphological features and biological behavior. There are 4 types: ependymoma, anaplastic ependymoma, myxopapillary ependymoma and subependymoma. (WHO) Undetermined C3017 Ependymoma WHO Grade II Ependymal Neoplasm || WHO Grade II Ependymal Tumor A WHO grade II, slow growing tumor of children and young adults, usually located intraventricularly. It is the most common ependymal neoplasm. It often causes clinical symptoms by blocking cerebrospinal fluid pathways. Key histological features include perivascular pseudorosettes and ependymal rosettes. (WHO) Undetermined C129351 Ependymoma, RELA Fusion-Positive An ependymoma characterized by gene fusions involving RELA, a transcription factor involved in NK-kB pathway activity. It accounts for the majority of supratentorial ependymomas in children. It has an unfavorable outcome when compared to other ependymoma subtypes. Malignant C7642 Epipodophyllotoxin-Related Myelodysplastic Syndrome Epipodophyllotoxin Related Myelodysplastic Syndrome A disorder seen following cancer chemotherapy. It typically manifests a few years after initiation of epipodophyllotoxin chemotherapy. Mutagenic potential of these non-intercalating DNA topoisomerase II inhibitors is believed to be increased with concurrent use of asparaginase or granulocyte colony-stimulating factor. Balanced translocations involving chromosomal bands 11q23 and 21q22 are commonly associated with this disorder. Clinical signs may include fatigue, dyspnea, bruising and frequent infections. Clinical course usually progresses to acute myeloid leukemia though most epipodophyllotoxin-related leukemias do not have an antecedent myelodysplastic phase. Prognosis is dismal with survivability usually less than one year. Malignant C9146 Epithelial Predominant Kidney Wilms Tumor Epithelial Predominant Kidney Adenosarcoma || Epithelial Predominant Kidney Nephroblastoma || Epithelial Predominant Renal Adenosarcoma || Epithelial Predominant Renal Wilm's Tumor || Epithelial Predominant Renal Wilms Tumor || Epithelial Predominant Renal Wilms' Tumor Wilms tumor of the kidney characterized by the predominance of the epithelial component. The epithelial cells may form papillary and tubular patterns and pseudorosettes. Malignant C4199 Epithelial-Myoepithelial Carcinoma A malignant neoplasm which occurs mostly in the major salivary glands (most frequently in the parotid gland), but also in the minor salivary glands of the oral mucosa and the tracheobronchial tree. It is characterized by the presence of ductal structures which are lined by an inner layer of cuboidal epithelial-type cells and an outer layer of myoepithelial cells with clear or eosinophilic cytoplasm. Malignant C4236 Epithelioid Cell Melanoma Epithelioid Cell Malignant Melanoma || Epithelioid Melanoma A melanoma characterized by the presence of malignant large epithelioid melanocytes. Malignant C3800 Epithelioid Hemangioendothelioma Epithelioid Angioendothelioma || Epithelioid Angiosarcoma A low-grade malignant blood vessel neoplasm. It is characterized by the presence of epithelioid endothelial cells. The neoplastic cells are arranged in cords and nests, which are embedded in a myxoid to hyalinized stroma. Malignant C3700 Epithelioid Leiomyosarcoma A morphologic variant of leiomyosarcoma characterized by the presence of epithelioid round cells with eosinophilic to clear cytoplasm. Malignant C6561 Epithelioid Malignant Peripheral Nerve Sheath Tumor Epithelioid MPNST || Malignant Epithelioid Neoplasm of Peripheral Nerve Sheath || Malignant Epithelioid Neoplasm of the Peripheral Nerve Sheath || Malignant Epithelioid Peripheral Nerve Sheath Neoplasm || Malignant Epithelioid Peripheral Nerve Sheath Tumor || Malignant Epithelioid Tumor of Peripheral Nerve Sheath || Malignant Epithelioid Tumor of the Peripheral Nerve Sheath A rare variant of malignant peripheral nerve sheath tumor composed predominantly or exclusively of epithelioid cells. Malignant C7985 Epithelioid Mesothelioma Epithelial Mesothelioma || Malignant Epithelial Mesothelioma A malignant neoplasm arising from mesothelial cells. It is characterized by the presence of cells with epithelioid morphology. The epithelioid cells usually have eosinophilic cytoplasm, bland nuclei, and form tubulopapillary, microglandular, or sheet-like patterns. Malignant C4025 Esophageal Adenocarcinoma Adenocarcinoma of Esophagus || Adenocarcinoma of the Esophagus || Esophagus Adenocarcinoma A malignant tumor with glandular differentiation arising predominantly from Barrett mucosa in the lower third of the esophagus. Rare examples of esophageal adenocarcinoma deriving from ectopic gastric mucosa in the upper esophagus have also been reported. Grossly, esophageal adenocarcinomas are similar to esophageal squamous cell carcinomas. Microscopically, adenocarcinomas arising in the setting of Barrett esophagus are typically papillary and/or tubular. The prognosis is poor. Malignant C3513 Esophageal Carcinoma Cancer of Esophagus || Cancer of the Esophagus || Carcinoma of Esophagus || Carcinoma of the Esophagus || Esophageal Cancer || Esophagus Carcinoma A malignant epithelial tumor arising from the esophageal mucosa. Two major histologic types of esophageal carcinoma have been described: squamous cell carcinoma and adenocarcinoma. This type of cancer is associated with excessive ethanol and cigarette usage. Malignant C95611 Esophageal Moderately Differentiated Squamous Cell Carcinoma An esophageal squamous cell carcinoma characterized by the presence of variable morphologic characteristics that include areas of prominent and poor keratinization. Malignant C95612 Esophageal Poorly Differentiated Squamous Cell Carcinoma An esophageal squamous cell carcinoma characterized by the presence of basal-like malignant squamous cells that form nests, often associated with central necrosis. Malignant C6762 Esophageal Small Cell Neuroendocrine Carcinoma Esophageal Small Cell Carcinoma || Esophageal Small Cell NEC || Esophagus Small Cell Carcinoma || Small Cell Carcinoma of Esophagus || Small Cell Carcinoma of the Esophagus || Small Cell Carcinoma, Esophagus An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the esophagus. It is characterized by the presence of malignant small cells. Malignant C4024 Esophageal Squamous Cell Carcinoma Esophageal Epidermoid Carcinoma || Esophageal SCC || Esophageal Squamous Cell Carcinoma, NOS || Esophageal Squamous Cell Carcinoma, Not Otherwise Specified || Esophagus SCC || Esophagus Squamous Cell Carcinoma || SCC of Esophagus || SCC of the Esophagus || Squamous Cell Carcinoma of Esophagus || Squamous Cell Carcinoma of the Esophagus A squamous cell carcinoma arising from the esophagus. It is associated with a long history of tobacco and alcohol abuse and is exceedingly rare before the age of 30. The median age is around 65 in both males and females. It is located mostly in the middle and lower third of the esophagus. Grossly, polypoid, ulcerated, plaque-like and occult lesions have been described. The microscopic features are the same as in other squamous cell carcinomas. Any degree of differentiation may occur, and variation within a single tumor is common. The prognosis is poor. Malignant C95610 Esophageal Well Differentiated Squamous Cell Carcinoma An esophageal squamous cell carcinoma characterized by the presence of prominent keratinization and low mitotic activity. Malignant C3407 Essential Thrombocythemia Essential Thrombocytemia || Essential Thrombocytosis || Idiopathic Thrombocythemia || Primary Thrombocythemia || Primary Thrombocytosis A chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. It is characterized by sustained thrombocytosis in the blood, increased numbers of large, mature megakaryocytes in the bone marrow, and episodes of thrombosis and/or hemorrhage. (WHO, 2008) Malignant C4817 Ewing Sarcoma ES || Ewing's Sarcoma || Ewing's Tumor A small round cell tumor that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing sarcoma/peripheral neuroectodermal tumor. It affects mostly males under age 20, and it can occur in soft tissue or bone. Pain and the presence of a mass are the most common clinical symptoms. Malignant C4835 Ewing Sarcoma of Bone Bone Ewing's Sarcoma || Ewing's Sarcoma of Bone || Ewing's Sarcoma, Osseous || Osseous Ewing's Sarcoma || Osseous Ewing's Tumor || Skeletal Ewing's Sarcoma || Skeletal Ewing's Tumor A small round cell bone tumor that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing sarcoma/peripheral neuroectodermal tumor. It often affects the diaphysis or metaphyseal-diaphyseal portion of long bones. Clinical findings include pain and a mass in the involved area. Fever, anemia, leukocytosis, and an increased sedimentation rate are often seen. X-ray examination reveals osteolytic lesions. The prognosis depends on the stage, anatomic location, and size of the tumor. Malignant C27291 Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Ewing Family of Tumors || Ewing's Family of Tumors || Ewing's Family of Tumours || Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor || Tumors of Ewing's Family || Tumors of the Ewing's Family A spectrum of malignant tumors, affecting mostly males under age 20, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. These tumors may occur in the soft tissues or the bones. Pain and the presence of a mass are the most common clinical symptoms. Malignant C35871 Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor of Bone Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor of Bone A spectrum of malignant tumors arising from the bone and characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. Pain and the presence of a mass are the most common clinical symptoms. Malignant C7453 Exocervical Carcinoma Carcinoma of Exocervix || Carcinoma of the Exocervix || Exocervix Carcinoma A carcinoma that arises from the squamous epithelium of the exocervix. Malignant C6081 External Ear Carcinoma Carcinoma of External Ear || Carcinoma of the External Ear A carcinoma that arises from the external ear. This category includes squamous cell carcinoma, basal cell carcinoma, and ceruminous adenocarcinoma. Malignant C49166 Extracardiac Rhabdomyoma A benign mesenchymal neoplasm that shows mature skeletal muscle differentiation. Benign C5437 Extracranial Neuroblastoma A neuroblastoma arising from an anatomic site other than the brain. Malignant C3918 Extragonadal Germ Cell Tumor Extragonadal Germ Cell Neoplasm || Extragonadal Germ Cell Neoplasms || Neoplasm of Extragonadal Germ Cell || Neoplasm of the Extragonadal Germ Cell || Primary Extragonadal Germ Cell Tumor || Tumor of Extragonadal Germ Cell || Tumor of the Extragonadal Germ Cell A germ cell tumor arising in an anatomic site other than the testis or ovary (e.g., central nervous system, lung, mediastinum, and retroperitoneum). Undetermined C7975 Extrahepatic Bile Duct Adenocarcinoma Adenocarcinoma of Extrahepatic Bile Duct || Adenocarcinoma of the Extrahepatic Bile Duct An adenocarcinoma arising from the epithelium of the extrahepatic bile ducts. Signs and symptoms include abdominal pain, anorexia, jaundice, pruritus, nausea and vomiting, and weight loss. Malignant C3860 Extrahepatic Bile Duct Carcinoma Carcinoma of Extrahepatic Bile Duct || Carcinoma of the Extrahepatic Bile Duct || Extrahepatic Bile Duct Cancer A carcinoma that arises from the extrahepatic bile ducts. The majority are adenocarcinomas. Malignant C3898 Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue || MALT Lymphoma || MALToma || Mucosa-Associated Lymphoid Tissue Lymphoma An indolent, extranodal type of non-Hodgkin lymphoma composed of small B-lymphocytes (centrocyte-like cells). The gastrointestinal tract is the most common site of involvement. Other common sites of involvement include lung, head and neck, ocular adnexae, skin, thyroid, and breast. Gastric involvement is associated with the presence of H. pylori infection. (WHO, 2001) Malignant C7848 Extraocular Retinoblastoma Metastatic Retinoblastoma Retinoblastoma that has spread beyond the eye e.g. to brain, soft tissue/bone, bone marrow. Malignant C4002 Extraosseous Plasmacytoma Extramedullary Plasmacytoma A plasma cell neoplasm arising at an extraosseous site. There is no involvement of the bone marrow. It most frequently involves the oropharynx, nasopharynx, sinuses, and larynx. Other sites of involvement include the gastrointestinal tract, central nervous system, breast, skin, lymph nodes, and bladder. A minority of patients have a monoclonal gammopathy. Treatment includes radiation therapy. Progression to plasma cell myeloma occurs in a minority of patients. Malignant C6586 Extrarenal Rhabdoid Tumor Malignant Extrarenal Rhabdoid Neoplasm || Rhabdoid Tumor of Soft Tissue A rhabdoid tumor which arises in the soft tissues. It occurs in infants and children and may be associated with loss of chromosome 22. It is characterized by the presence of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. Malignant C7135 Extraskeletal Ewing Sarcoma Extra-Osseous Ewing's Sarcoma || Extraosseous Ewing Sarcoma || Extraosseous Ewing's Sarcoma || Extraosseous Ewing's Tumor || Extraskeletal Ewing's Sarcoma A rare malignant neoplasm of the soft tissues. It is typically a disease of children and young adults. Most commonly occurs in the paravertebral region, chest wall, pelvis and lower extremities. Treatment includes local excision with consideration for post-operative chemotherapy and/or radiotherapy. Malignant C27293 Extraskeletal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor || Extraosseous Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor A spectrum of malignant tumors arising from the soft tissues, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. Pain and the presence of a mass are the most common clinical symptoms. Malignant C8810 Extraskeletal Osteosarcoma Extraosseous Osteosarcoma || Extraskeletal Osteogenic Sarcoma || Soft Tissue Osteosarcoma An osteosarcoma arising from the soft tissue. Malignant C92555 Extraventricular Neurocytoma Extraventricular Neurocytoma (WHO Grade II) A WHO grade II neoplasm that arises from the brain parenchyma. Morphologically it is characterized by the presence of neoplastic uniform, round cells with neuronal differentiation. Unlike central neurocytoma, it does not involve the lateral ventricles. It usually affects young adults and has a favorable prognosis. Undetermined C6079 Eye Carcinoma Carcinoma of Eye || Carcinoma of the Eye || Ocular Carcinoma A carcinoma that arises from any of the structures of the eye. Malignant C6078 Eyelid Carcinoma Carcinoma of Eyelid || Carcinoma of the Eyelid A carcinoma that arises from the eyelid. Examples include basal cell carcinoma and squamous cell carcinoma. Malignant C6265 Fallopian Tube Adenocarcinoma Adenocarcinoma of Fallopian Tube || Adenocarcinoma of the Fallopian Tube An adenocarcinoma that arises from the fallopian tube. Histologic subtypes include clear cell, endometrioid, serous, and mucinous adenocarcinoma. It spreads to adjacent organs, regional lymph nodes, and peritoneum. Malignant C3867 Fallopian Tube Carcinoma Cancer of Fallopian Tube || Cancer of the Fallopian Tube || Carcinoma of Fallopian Tube || Carcinoma of the Fallopian Tube || Fallopian Tube Cancer A carcinoma arising from the fallopian tube. Most patients are postmenopausal, and postmenopausal bleeding is the most frequently seen symptom. Morphologically, the majority of fallopian tube carcinomas are serous or endometrioid adenocarcinomas. Malignant C6282 Fallopian Tube Squamous Cell Carcinoma Squamous Cell Carcinoma of Fallopian Tube || Squamous Cell Carcinoma of the Fallopian Tube A rare squamous cell carcinoma that arises from the fallopian tube. Malignant C115225 Familial Neuroblastoma Hereditary Neuroblastoma Genetic inheritance of neuroblastoma caused by mutations in the ALK or PHOX2B genes. Familial neuroblastomas have a higher incidence of multiple primary tumors and are diagnosed at an earlier age. Malignant C118829 Familial Nonmedullary Thyroid Gland Carcinoma Familial Non-medullary Thyroid Gland Carcinoma || Non-Syndromic Familial Thyroid Cancer A papillary or follicular thyroid gland carcinoma with a genetic component that develops within the same family. Current studies suggest that it is inherited in an autosomal dominant pattern. It is often multifocal and bilateral and usually affects younger patients. Malignant C115212 Familial Waldenstrom Macroglobulinemia Familial Waldenström Macroglobulinemia Waldenstrom macroglobulinemia in a patient who has at least one first degree relative with either Waldenstrom macroglobulinemia or another B-cell lymphoproliferative disorder. Malignant C2918 Female Breast Carcinoma Breast Carcinoma, Female || Carcinoma of Female Breast || Carcinoma of the Female Breast || Female Breast Cancer || Mammary Carcinoma of Female Breast || Mammary Carcinoma of the Female Breast A carcinoma that arises from the breast in females. It is the most common malignant tumor that affects females. Malignant C4322 Fibrillary Astrocytoma The most frequent histological variant of diffuse astrocytoma. It is predominantly composed of fibrillary neoplastic astrocytes. Nuclear atypia is a diagnostic criterion but mitotic activity, necrosis and microvascular proliferation are absent. The occasional or regional occurrence of gemistocytic neoplastic cells is compatible with the diagnosis of fibrillary astrocytoma. (WHO) Undetermined C4020 Fibroblastic Osteosarcoma Fibroblastic Osteogenic Sarcoma || Fibrosarcomatous Osteogenic Sarcoma || Fibrosarcomatous Osteosarcoma A conventional osteosarcoma characterized by the presence of spindle shaped cells. Malignant C81758 Fibroblastic Reticular Cell Tumor A very rare dendritic cell tumor affecting the lymph nodes, spleen, and soft tissues. Morphologically it is similar to the interdigitating dendritic cell sarcoma or follicular dendritic cell sarcoma. The tumor cells are positive for cytokeratin and CD68. Clinical outcome is variable. Malignant C4131 Fibrolamellar Carcinoma Fibrolamellar Carcinoma of Liver Cells || Fibrolamellar Carcinoma of the Liver Cells || Fibrolamellar Hepatocellular Carcinoma || Hepatocellular Fibrolamellar Carcinoma || Liver Cell Fibrolamellar Carcinoma || Oncocytic Hepatocellular Tumor || Polygonal Cell Type Hepatocellular Carcinoma with Fibrous Stroma A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and is seen predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma. The prognosis is similar to classical hepatocellular carcinoma that arises in non-cirrhotic livers, and better than hepatocellular carcinoma that arises in cirrhotic livers. Malignant C3043 Fibrosarcoma A malignant mesenchymal fibroblastic neoplasm affecting the soft tissue and bone. Malignant C3739 Fibrous Histiocytoma Benign Fibrous Histiocytoma A benign neoplasm composed of fibroblastic spindle cells in a whorled storiform pattern. It is characterized by the presence of foam cells, inflammatory cells, hemosiderin deposition and stromal hemorrhage. Benign C4330 Fibrous Meningioma Fibroblastic Meningioma A WHO grade I meningioma characterized by the presence of spindle cells that form bundles in a collagen matrix. Undetermined C65193 Flat Adenoma GI Tract Flat Adenoma || Gastrointestinal Tract Flat Adenoma An adenoma of the gastrointestinal tract mucosa which grossly and morphologically does not appear as an elevated or polypoid lesion. Benign C4041 Floor of Mouth Squamous Cell Carcinoma Floor of Mouth SCC || SCC of Floor of Mouth || SCC of the Floor of Mouth || Squamous Cell Carcinoma of Floor of Mouth || Squamous Cell Carcinoma of the Floor of Mouth A squamous cell carcinoma of the oral cavity that arises from the floor of the mouth. Malignant C9281 Follicular Dendritic Cell Sarcoma Follicular Dendritic Cell Sarcoma/Tumor A neoplasm composed of spindle to ovoid cells which have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It usually behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy. Recurrences have been reported in up to half of the cases. Malignant C3209 Follicular Lymphoma Follicle Center Lymphoma || Follicular Non-Hodgkin Lymphoma || Follicular Non-Hodgkin's Lymphoma A neoplasm of follicle centre B cells which has at least a partial follicular pattern. Follicular lymphomas comprise about 35% of adult non-Hodgkin lymphomas in the U.S. and 22% worldwide. Most patients have widespread disease at diagnosis. Morphologically, follicular lymphomas are classified as Grade 1, Grade 2, and Grade 3, depending on the percentage of the large lymphocytes present. The vast majority of cases (70-95%) express the BCL-2 rearrangement [t(14;18)]. Histological grade correlates with prognosis. Grades 1 and 2 follicular lymphomas are indolent and grade 3 is more aggressive (adapted from WHO, 2001). Malignant C80375 Follicular T-Cell Lymphoma Follicular Variant Peripheral T-Cell Lymphoma A lymph node-based peripheral T-cell lymphoma of T-follicular helper (TFH) cells, with a predominantly follicular growth pattern. It lacks characteristic features of angioimmunoblastic T-cell lymphoma such as proliferation of high endothelial venules or extrafollicular follicular dendritic cells. (WHO 2017) Malignant C121152 FSH-Producing Gonadotroph Adenoma FSH-Producing Pituitary Gland Adenoma || Follicle Stimulating Hormone-Producing Gonadotroph Adenoma An adenoma of the anterior lobe of the pituitary gland that produces follicle-stimulating hormone (FSH). Benign C8388 Functioning Pituitary Gland Adenoma Functioning Adenoma of Pituitary || Functioning Adenoma of Pituitary Gland || Functioning Adenoma of the Pituitary || Functioning Adenoma of the Pituitary Gland || Functioning Pituitary Adenoma || Functioning Pituitary Gland Adenoma (Benign) || Secretory Adenoma of Pituitary || Secretory Adenoma of Pituitary Gland || Secretory Adenoma of the Pituitary || Secretory Adenoma of the Pituitary Gland || Secretory Pituitary Adenoma || Secretory Pituitary Gland Adenoma A hormone producing pituitary gland adenoma, associated with a hormonal syndrome. Benign C123736 Fusion-Negative Rhabdomyosarcoma A rhabdomyosarcoma characterized by the absence of chromosomal translocation t(1;13)(p36;q14) or t(2;13)(q35;q14) and therefore the absence of PAX7-FOXO1 or PAX3-FOXO1 gene fusion. Malignant C123735 Fusion-Positive Rhabdomyosarcoma A rhabdomyosarcoma characterized by the presence of chromosomal translocation t(1;13)(p36;q14) that results in PAX7-FOXO1 gene fusion; or translocation t(2;13)(q35;q14) that results in PAX3-FOXO1 gene fusion. Malignant C9166 Gallbladder Adenocarcinoma Adenocarcinoma of Gallbladder || Adenocarcinoma of the Gallbladder An adenocarcinoma arising from the gallbladder. It is the most common malignant tumor of the gallbladder and it is usually well to moderately differentiated. The incidence is higher in patients with gallstones than in patients without gallstones. Signs and symptoms usually present late in the course of the disease and are reminiscent of those of chronic cholecystitis including right upper quadrant pain. Histologic variants include adenocarcinoma of the intestinal type, clear cell adenocarcinoma, mucinous adenocarcinoma, papillary adenocarcinoma, and signet ring adenocarcinoma. Malignant C7720 Gallbladder Adenoma Adenoma of Gallbladder || Adenoma of the Gallbladder A polypoid epithelial neoplasm that arises from the gallbladder. According to the neoplastic growth pattern, it is classified as tubular, tubulopapillary, or papillary. Benign C7356 Gallbladder Adenosquamous Carcinoma Adenosquamous Carcinoma of Gallbladder || Adenosquamous Carcinoma of the Gallbladder A carcinoma that arises from the gallbladder. It is characterized by the presence of glandular and squamous malignant epithelial components. Malignant C3844 Gallbladder Carcinoma Cancer of Gallbladder || Cancer of the Gallbladder || Carcinoma of Gallbladder || Carcinoma of the Gallbladder || Gallbladder Cancer A malignant tumor arising from the epithelium of the gallbladder. It is usually associated with the presence of gallstones. Clinical symptoms are not specific and usually present late in the course. Morphologically, most gallbladder carcinomas are adenocarcinomas; squamous cell carcinomas, adenosquamous carcinomas, signet ring carcinomas, and undifferentiated carcinomas can also occur. Malignant C5743 Gallbladder Intracholecystic Papillary Neoplasm with an Associated Invasive Carcinoma Gallbladder ICPN with an Associated Invasive Carcinoma || Gallbladder Intracholecystic Papillary (Tubular) Neoplasm with an Associated Invasive Carcinoma || Gallbladder Intracystic/Intaductal Papillary Neoplasm with an Associated Invasive Carcinoma || Intracystic Papillary Neoplasm with an Associated Invasive Carcinoma An intracholecystic papillary neoplasm, usually with high grade intraepithelial neoplasia, that arises from the gallbladder. It is associated with the presence of an invasive carcinoma. The carcinomatous component is an adenocarcinoma. Malignant C6763 Gallbladder Small Cell Neuroendocrine Carcinoma Gallbladder Small Cell Carcinoma || Gallbladder Small Cell NEC || Small Cell Carcinoma of Gallbladder || Small Cell Carcinoma of the Gallbladder An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the gallbladder. It is characterized by the presence of malignant small cells. Malignant C9170 Gallbladder Squamous Cell Carcinoma Epidermoid Carcinoma of Gallbladder || Epidermoid Carcinoma of the Gallbladder || Epidermoid Gallbladder Carcinoma || Squamous Cell Carcinoma of Gallbladder || Squamous Cell Carcinoma of the Gallbladder || Squamous Cell Gallbladder Carcinoma A carcinoma that arises from the gallbladder. It is composed entirely by malignant squamous epithelial cells. Malignant C3083 Gamma Heavy Chain Disease Franklin Disease || Franklin's Disease A clonal disorder characterized by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course is typically more aggressive than in lymphoplasmacytic lymphoma. Malignant C6934 Gangliocytoma A well differentiated, slow growing neuroepithelial neoplasm composed of neoplastic, mature ganglion cells. Undetermined C3788 Ganglioglioma A well differentiated, slow growing neuroepithelial neoplasm composed of neoplastic, mature ganglion cells and neoplastic glial cells. Some gangliogliomas show anaplastic features in their glial component and are considered to be WHO grade III. Rare cases of newly diagnosed gangliogliomas with grade IV (glioblastoma) changes in the glial component have also been reported. (Adapted from WHO) Undetermined C3790 Ganglioneuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, ganglion cells, and a stroma with Schwannian differentiation constituting more than fifty-percent of the tumor volume. There are two histologic subtypes identified: ganglioneuroblastoma, intermixed and ganglioneuroblastoma, nodular. Malignant C42057 Ganglioneuroblastoma, Intermixed Ganglioneuroblastoma, Intermixed (Schwannian Stroma-Rich) A ganglioneuroblastoma characterized by the presence of neuroblastic cells in a Schwannian stroma, without the presence of hemorrhagic neuroblastic nodules. Malignant C42058 Ganglioneuroblastoma, Nodular A ganglioneuroblastoma characterized by the presence of neuroblastic cells in a Schwannian stroma, and the formation of hemorrhagic neuroblastic nodules. Malignant C3049 Ganglioneuroma Ganglioneuroma (Schwannian Stroma-Dominant) A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic trunk in the mediastinum. Histologic features include spindle cell proliferation (resembling a neurofibroma) and the presence of large ganglion cells. Common presenting features include a palpable abdominal mass, hepatomegaly, and a thoracic mass detected on routine chest X-ray. Benign C4004 Gastric Adenocarcinoma Adenocarcinoma of Stomach || Adenocarcinoma of the Stomach || Stomach Adenocarcinoma An adenocarcinoma arising from the stomach glandular epithelium. Gastric adenocarcinoma is primarily a disease of older individuals. It most commonly develops after a long period of atrophic gastritis and is strongly associated with Helicobacter pylori infection. The lack of early symptoms often delays the diagnosis of gastric cancer. The majority of patients present with advanced tumors which have poor rates of curability. Microscopically, two important histologic types of gastric adenocarcinoma are recognized: the intestinal and diffuse type. The overall prognosis of gastric adenocarcinomas is poor, even in patients who receive a "curative" resection (adapted from Sternberg's Surgical Pathology, 3rd ed., 1999). Malignant C7699 Gastric Adenoma Adenoma of Stomach || Adenoma of the Stomach A neoplastic polyp that arises from the stomach. This category includes intestinal-type adenomatous polyps, gastric-type adenomas, and fundic gland polyps. Benign C5474 Gastric Adenosquamous Carcinoma Adenosquamous Carcinoma of Stomach || Adenosquamous Carcinoma of the Stomach A carcinoma that arises from the stomach and is characterized by the presence of malignant cells with glandular and squamous differentiation. Malignant C4911 Gastric Carcinoma Cancer of Stomach || Cancer of the Stomach || Carcinoma of Stomach || Carcinoma of the Stomach || Gastric Cancer || Stomach Cancer || Stomach Carcinoma A malignant epithelial tumor of the stomach mucosa. The vast majority of gastric carcinomas are adenocarcinomas, arising from the gastric glandular epithelium. Malignant C9159 Gastric Diffuse Adenocarcinoma Adenocarcinoma of Linitis Plastica Type || Adenocarcinoma of the Linitis Plastica Type || Diffuse Adenocarcinoma of Stomach || Diffuse Adenocarcinoma of the Stomach || Diffuse Stomach Adenocarcinoma An adenocarcinoma arising from the stomach. Microscopically, it is characterized by the presence of a diffuse infiltrate, composed of individual adenocarcinoma cells or groups of adenocarcinoma cells in a fibrous or mucoid stroma. Many cells contain mucin droplets, producing a signet-ring configuration. Malignant C9157 Gastric Intestinal-Type Adenocarcinoma Intestinal Adenocarcinoma of Stomach || Intestinal Adenocarcinoma of the Stomach || Intestinal Gastric Adenocarcinoma || Intestinal Stomach Adenocarcinoma An adenocarcinoma of the stomach arising on a background of intestinal metaplasia. Microscopically, it is characterized by a glandular pattern and it closely resembles a colonic adenocarcinoma. Grossly, it tends to be nodular, polypoid or ulcerated. Malignant C95763 Gastric Intramucosal Adenocarcinoma Gastric Intramucosal Carcinoma || Gastric Intramucosal Invasive Neoplasia || Gastric Intramucosal Invasive Neoplasia/ Intramucosal Carcinoma A neoplasm that arises from the gastric glandular epithelium and invades the lamina propria but not the submucosa. There is marked glandular crowding, branching and budding, intraluminal necrosis, and prominent mitotic activity. Desmoplastic changes may be present or absent. Malignant C5248 Gastric Mucinous Adenocarcinoma Mucinous Adenocarcinoma of Stomach || Mucinous Adenocarcinoma of the Stomach || Mucinous Gastric Adenocarcinoma || Mucinous Stomach Adenocarcinoma A variant of gastric adenocarcinoma with more than half of the tumor containing extracellular mucinous pools. Malignant C5266 Gastric Mucosa-Associated Lymphoid Tissue Lymphoma Gastric MALT Lymphoma || Gastric MALToma || MALT Lymphoma of Stomach || MALT Lymphoma of the Stomach || MALToma of Stomach || MALToma of the Stomach || Primary Gastric B-Cell MALT Lymphoma || Primary Gastric MALT Lymphoma || Primary MALT Lymphoma of Stomach || Primary MALT Lymphoma of the Stomach A low grade, indolent B-cell lymphoma, usually associated with Helicobacter pylori infection. Morphologically it is characterized by a dense mucosal atypical lymphocytic (centrocyte-like cell) infiltrate with often prominent lymphoepithelial lesions and plasmacytic differentiation. Approximately 40% of gastric MALT lymphomas carry the t(11;18)(q21;q21). Such cases are resistant to Helicobacter pylori therapy. Malignant C95743 Gastric Poorly Cohesive Adenocarcinoma Gastric Poorly Cohesive Carcinoma || Poorly Cohesive Gastric Adenocarcinoma || Poorly Cohesive Gastric Carcinoma An adenocarcinoma that arises from the gastric mucosa and is characterized by the presence of isolated malignant cells or malignant cells that form small aggregates. This category includes signet ring cell adenocarcinomas, adenocarcinomas that are composed of malignant cells resembling lymphocytes and histiocytes, and adenocarcinomas that are composed of malignant cells with deeply eosinophilic cytoplasm. Malignant C5250 Gastric Signet Ring Cell Adenocarcinoma Gastric Signet Ring Cell Adenocarcinoma || Gastric Signet-Ring Cell Adenocarcinoma || Signet Ring Cell Adenocarcinoma of Stomach || Signet Ring Cell Adenocarcinoma of the Stomach || Signet Ring Cell Gastric Adenocarcinoma || Signet Ring Cell Stomach Adenocarcinoma A poorly cohesive gastric adenocarcinoma characterized by malignant cells containing intracytoplasmic mucin. Malignant C6764 Gastric Small Cell Neuroendocrine Carcinoma Gastric Small Cell Carcinoma || Small Cell Carcinoma of Stomach || Small Cell Carcinoma of the Stomach An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the stomach. It is characterized by the presence of malignant small cells. Malignant C5475 Gastric Squamous Cell Carcinoma Squamous Cell Carcinoma of Stomach || Squamous Cell Carcinoma of the Stomach A rare carcinoma of the stomach resembling squamous cell carcinomas arising elsewhere in the body. Malignant C5476 Gastric Undifferentiated Carcinoma Anaplastic Carcinoma of Stomach || Anaplastic Carcinoma of the Stomach || Anaplastic Gastric Carcinoma || Undifferentiated Carcinoma of Stomach || Undifferentiated Carcinoma of the Stomach || Undifferentiated Gastric Carcinoma A carcinoma that arises from the stomach and is characterized by the absence of microscopic features of glandular or squamous differentiation. Malignant C9296 Gastroesophageal Junction Adenocarcinoma Adenocarcinoma of Cardioesophageal Junction || Adenocarcinoma of Gastroesophageal Junction || Adenocarcinoma of the Cardioesophageal Junction || Adenocarcinoma of the EG Junction || Adenocarcinoma of the Esophagogastric Junction || Adenocarcinoma of the GE Junction || Adenocarcinoma of the Gastroesophageal Junction An adenocarcinoma that arises from and straddles the junction of the stomach and esophagus. The category of adenocarcinomas of the gastroesophageal junction also includes the majority of adenocarcinomas previously called gastric cardia adenocarcinomas. Squamous cell carcinomas that affect or cross the junction of the stomach and esophagus are classified as carcinomas of the distal esophagus. Adenocarcinoma of the gastroesophageal junction occurs more often in Caucasian middle aged and elderly males. Clinical signs and symptoms include dysphagia, abdominal pain, and weight loss. The prognosis depends on the completeness of the surgical resection, the number of lymph nodes involved by cancer, and the presence or absence of postoperative complications. The presence of TP53 mutations indicates worse prognosis. Malignant C3868 Gastrointestinal Stromal Tumor Gastrointestinal Stromal Neoplasm A stromal tumor most commonly seen in the gastrointestinal tract. Rare cases of solitary masses in the omentum or the mesentery have also been reported (extragastrointestinal gastrointestinal stromal tumor). It is a tumor that differentiates along the lines of interstitial cells of Cajal. Most cases contain KIT- or PDGFRA-activating mutations. Until recently, surgery has been the only effective therapy for this tumor. However, many patients still experience recurrence. Conventional chemotherapy and radiation therapy have been of limited value. A KIT tyrosine kinase inhibitor, imatinib mesylate (also known as STI-571 or Gleevec), is now effective in the treatment of relapsed and unresectable cases. Undetermined C54000 Gastrointestinal Stromal Tumor of Uncertain Malignant Potential A gastrointestinal stromal tumor that is characterized by a maximum diameter greater than 5 cm and equal or less than 10 cm (gastric localization), or greater than 2 cm and equal or less than 5 cm (intestinal localization) and no more than 5 mitotic figures per 50 high power fields. Uncertain Malignant Potential C4321 Gemistocytic Astrocytoma Gemistocytoma A rare variant of diffuse astrocytoma. It is characterized by the presence of a conspicuous, though variable, fraction of gemistocytic neoplastic astrocytes. Gemistocytes are round to oval astrocytes with abundant, glassy, non-fibrillary cytoplasm which appears to displace the dark, angulated nucleus to the periphery of the cell. To make the diagnosis of gemistocytic astrocytoma, gemistocytes should amount to more than approximately 20% of all tumor cells. (Adapted from WHO) Undetermined C129272 Gemistocytic Astrocytoma, IDH-Mutant Gemistocytic astrocytoma carrying IDH mutations. Undetermined C3708 Germ Cell Tumor Germ Cell Neoplasm || Neoplasm of Germ Cell || Neoplasm of the Germ Cell || Tumor of Germ Cell || Tumor of the Germ Cell A benign or malignant, gonadal or extragonadal neoplasm that originates from germ cells. Representative examples include teratoma, seminoma, embryonal carcinoma, and yolk sac tumor. Undetermined C4646 Gestational Choriocarcinoma A rare, highly malignant epithelial tumor that develops from a hydatidiform mole (50%), following abortion (25%), or during normal pregnancy (22%). It is characterized by the presence of invasive and anaplastic trophoblastic tissue composed of intermediate trophoblastic cells, cytotrophoblasts and syncytiotrophoblasts, abundant vascularity, and secretion of human chorionic gonadotropin. Because of rapid growth and a high propensity for hemorrhage, this neoplasm often constitutes a medical emergency. Malignant C4699 Gestational Trophoblastic Tumor A diverse group of pregnancy-related tumors characterized by excessive proliferation of trophoblasts. It includes gestational choriocarcinoma, epithelioid trophoblastic tumor, placental site trophoblastic tumor, and mixed trophoblastic tumor. Undetermined C3779 Giant Cell Carcinoma Carcinoma, Giant Cell A malignant epithelial neoplasm composed of giant, pleomorphic cells. Malignant C4700 Giant Cell Fibroblastoma A rare, locally aggressive fibroblastic neoplasm typically affecting young patients (predominantly boys). It manifests with painless nodules in the dermis or subcutaneous tissues. Morphologically, it contains giant cells and wide vessel-like spaces. This tumor can recur but metastases have not been reported. Undetermined C4325 Giant Cell Glioblastoma A rare histological variant of glioblastoma (WHO grade IV) with a predominance of bizarre, multinucleated giant cells, an occasionally abundant stromal reticulin network, and a high frequency of TP53 mutations. (WHO) Malignant C3055 Giant Cell Tumor A benign, intermediate, or malignant tumor that arises from the bone or soft tissue. It is characterized by the presence of multinucleated osteoclast-like giant cells. Undetermined C49107 Giant Cell Tumor of Soft Tissue A painless, well circumscribed tumor arising in soft tissue, usually of the upper and lower extremities. Morphologically, it is characterized by a multinodular growth pattern. The cellular infiltrate is composed of mononuclear round or oval cells and multinucleated osteoclast-like giant cells, in a rich vascular stroma. It rarely metastasizes. Undetermined C129289 Gingival Spindle Cell Carcinoma A poorly differentiated squamous cell carcinoma that arises from the gingiva. It is characterized by the presence of malignant pleomorphic spindle cells. Malignant C129857 Gingival Squamous Cell Carcinoma Gingival SCC A squamous cell carcinoma that arises from the gingival mucosa. It presents as an ulcerated lesion or exophytic mass. The prognosis is usually poor. Malignant C6560 Glandular Malignant Peripheral Nerve Sheath Tumor Glandular MPNST || Malignant Glandular Neoplasm of Peripheral Nerve Sheath || Malignant Glandular Neoplasm of the Peripheral Nerve Sheath || Malignant Glandular Peripheral Nerve Sheath Neoplasm || Malignant Glandular Peripheral Nerve Sheath Tumor || Malignant Glandular Schwannoma || Malignant Glandular Tumor of Peripheral Nerve Sheath || Malignant Glandular Tumor of the Peripheral Nerve Sheath A variant of malignant peripheral nerve sheath tumor characterized by the presence of glandular epithelium. Malignant C3058 Glioblastoma GBM (Glioblastoma) || Glioblastoma Multiforme || Grade IV Astrocytic Neoplasm || Grade IV Astrocytic Tumor || Spongioblastoma Multiforme || WHO Grade IV Glioma The most malignant astrocytic tumor (WHO grade IV). It is composed of poorly differentiated neoplastic astrocytes and it is characterized by the presence of cellular polymorphism, nuclear atypia, brisk mitotic activity, vascular thrombosis, microvascular proliferation and necrosis. It typically affects adults and is preferentially located in the cerebral hemispheres. It may develop from diffuse astrocytoma WHO grade II or anaplastic astrocytoma (secondary glioblastoma, IDH-mutant), but more frequently, it manifests after a short clinical history de novo, without evidence of a less malignant precursor lesion (primary glioblastoma, IDH- wildtype). (Adapted from WHO) Malignant C111691 Glioblastoma by Gene Expression Profile Classification of glioblastoma into molecular subtypes as defined by gene expression profiling. Malignant C39750 Glioblastoma, IDH-Wildtype Primary Glioblastoma || Primary Glioblastoma Multiforme || Primary Glioblastoma, IDH-Wildtype A glioblastoma that arises de novo. It is more commonly seen in older patients. Mutations in IDH1 or IDH2 genes are not present. Malignant C129295 Glioblastoma, Not Otherwise Specified Glioblastoma, NOS Glioblastoma in which there is insufficient information on the IDH genes status. Malignant C5419 Gliofibroma An astrocytic tumor affecting young people. Morphologically, it is characterized by the presence of collagenous tissue surrounding neoplastic astrocytes. In some cases the collagen is produced by the tumor cells (desmoplastic astrocytoma), whereas in others it is produced by mesenchymal cells (mixed glioma/fibroma). Undetermined C3059 Glioma Glial Neoplasm || Glial Tumor || Glioma || Neoplasm of Neuroglia || Neoplasm of the Neuroglia || Neuroglial Neoplasm || Neuroglial Tumor || Tumor of Neuroglia || Tumor of the Neuroglia A benign or malignant brain and spinal cord tumor that arises from glial cells (astrocytes, oligodendrocytes, ependymal cells). Tumors that arise from astrocytes are called astrocytic tumors or astrocytomas. Tumors that arise from oligodendrocytes are called oligodendroglial tumors. Tumors that arise from ependymal cells are called ependymomas. Undetermined C4318 Gliomatosis Cerebri Astrocytosis cerebri || Gliomatosis A diffuse glial tumor which infiltrates the brain extensively, involving more than two lobes. It is frequently bilateral and often extends to the infratentorial structures, even to the spinal cord. It is probably of astrocytic origin, although GFAP expression may be scant or absent. (Adapted from WHO.) Malignant C92550 Glioneuronal Tumor with Neuropil-Like Islands A rare, WHO grade II or III infiltrating astrocytoma characterized by the presence of sharply demarcated foci, composed of a neuropil-like matrix. Undetermined C3796 Gliosarcoma Glioblastoma with a Sarcomatous Component A rare histological variant of glioblastoma (WHO grade IV) characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation (WHO). Malignant C3060 Glomus Tumor Glomus Neoplasm A rare benign or malignant mesenchymal neoplasm arising from cells that resemble the modified smooth muscle cells of the glomus body. The majority of glomus tumors occur in the distal extremities. Undetermined C8186 Glottic Squamous Cell Carcinoma Epidermoid Carcinoma of Glottis || Epidermoid Carcinoma of the Glottis || Glottic Epidermoid Carcinoma || Glottis Epidermoid Carcinoma || Glottis Squamous Cell Carcinoma || Squamous Cell Carcinoma of Glottis || Squamous Cell Carcinoma of the Glottis A squamous cell carcinoma of the larynx that arises from the glottic area. It may remain localized for a long period then in late disease stage, it may spread to the opposite true vocal cord, supraglottic and subglottic areas, and the soft tissues of the neck. Hoarseness is the presenting symptom. Malignant C66776 Gonadal Polyembryoma A rare malignant germ cell tumor arising from the testis and less often from the ovary. It is characterized by the presence of embryoid bodies which resemble early embryos. Malignant C3754 Gonadoblastoma A mixed germ cell/sex cord-stromal tumor characterized by the presence of large germ cells which resemble seminoma cells and small cells which resemble Sertoli or granulosa cells. It occurs in the testis and the ovary and is identified in children and adults. It is often associated with gonadal dysgenesis and abnormal karyotype. Undetermined C45915 Gonadotroph Adenoma Gonadotrope Adenoma || Gonadotroph Cell Adenoma || Gonadotropin Producing Pituitary Gland Adenoma || Gonadotropin-Producing Pituitary Gland Adenoma || Gonadotropin-Secreting Pituitary Gland Adenoma || Gonadotropinoma An adenoma of the anterior lobe of the pituitary gland that produces gonadotrophic hormones (FSH and/or LH) or shows evidence towards gonadotroph differentiation. The majority of cases are hormonally non-functional. Clinical manifestations include visual disturbances, hypopituitarism, headache, and acute hemorrhagic necrosis of the pituitary gland. Benign C9452 Grade 1 Colon Adenocarcinoma Grade I Colon Adenocarcinoma || Well Differentiated Colon Adenocarcinoma A colon adenocarcinoma characterized by the presence of a malignant cellular infiltrate with more than 95% glandular formation. Malignant C9446 Grade 1 Colorectal Adenocarcinoma Grade I Colorectal Adenocarcinoma || Low Grade Colorectal Adenocarcinoma || Well Differentiated Colorectal Adenocarcinoma A colorectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with more than 95% glandular formation. Malignant C3465 Grade 1 Follicular Lymphoma Follicular Lymphoma Grade 1 || Grade I Follicular Lymphoma || WHO Follicular Lymphoma Histologic Grade 1 A follicular lymphoma which contains up to 5 centroblasts per 40X high-power microscopic field. Malignant C9451 Grade 1 Rectal Adenocarcinoma Grade I Rectal Adenocarcinoma || Well Differentiated Rectal Adenocarcinoma A rectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with more than 95% glandular formation. Malignant C9453 Grade 2 Colon Adenocarcinoma Grade II Colon Adenocarcinoma || Moderately Differentiated Colon Adenocarcinoma A colon adenocarcinoma characterized by the presence of a malignant cellular infiltrate with 50-95% glandular formation. Malignant C9447 Grade 2 Colorectal Adenocarcinoma Grade II Colorectal Adenocarcinoma || Low Grade Colorectal Adenocarcinoma || Moderately Differentiated Colorectal Adenocarcinoma A colorectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with 50-95% glandular formation. Malignant C8968 Grade 2 Follicular Lymphoma Follicular Lymphoma Grade 2 || Follicular Mixed Cell Lymphoma || Follicular Mixed Lymphocytic-Histiocytic Lymphoma || Grade II Follicular Lymphoma || Grade II Follicular Mixed Cell Lymphoma || Nodular Mixed Lymphoma || WHO Follicular Lymphoma Histologic Grade 2 A follicular lymphoma which contains 6-15 centroblasts per 40X high-power microscopic field. Malignant C9450 Grade 2 Rectal Adenocarcinoma Grade II Rectal Adenocarcinoma || Moderately Differentiated Rectal Adenocarcinoma A rectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with 50-95% glandular formation. Malignant C9454 Grade 3 Colon Adenocarcinoma Grade III Colon Adenocarcinoma || Poorly Differentiated Colon Adenocarcinoma A colon adenocarcinoma characterized by the presence of a malignant cellular infiltrate with less than 50% glandular formation. Malignant C9448 Grade 3 Colorectal Adenocarcinoma Grade III Colorectal Adenocarcinoma || High Grade Colorectal Adenocarcinoma || Poorly Differentiated Colorectal Adenocarcinoma A colorectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with less than 50% glandular formation. Malignant C3460 Grade 3 Follicular Lymphoma Follicular Large Cell Lymphoma || Follicular Lymphoma Grade 3 || Grade III Follicular Large Cell Lymphoma || Grade III Follicular Lymphoma || WHO Follicular Lymphoma Histologic Grade 3 A follicular lymphoma which contains more than 15 centroblasts per 40X high-power microscopic field. Malignant C9449 Grade 3 Rectal Adenocarcinoma Grade III Rectal Adenocarcinoma || Poorly Differentiated Rectal Adenocarcinoma A rectal adenocarcinoma characterized by the presence of a malignant cellular infiltrate with less than 50% glandular formation. Malignant C7931 Grade I Lymphomatoid Granulomatosis Angiocentric Immunoproliferative Lesion Grade I || Grade I Angiocentric Immunoproliferative Lesion || Grade I Angiocentric Immunoproliferative Lesions Lymphomatoid granulomatosis characterized by the presence of a polymorphous lymphoid infiltrate without cytologic atypia. Large lymphocytes are absent or rare. By in situ hybridization, EBV-positive cells are infrequently seen. Uncertain Malignant Potential C38936 Grade I Meningioma Grade 1 Meningioma || WHO Grade I Meningioma A benign meningioma which may recur in approximately 7-20% of the cases. This category includes the angiomatous meningioma, fibrous meningioma, lymphoplasmacyte-rich meningioma, meningothelial meningioma, metaplastic meningioma, microcystic meningioma, psammomatous meningioma, secretory meningioma, and transitional meningioma. Undetermined C7932 Grade II Lymphomatoid Granulomatosis Angiocentric Immunoproliferative Lesion Grade II || Grade II Angiocentric Immunoproliferative Lesion || Grade II Angiocentric Immunoproliferative Lesions Lymphomatoid granulomatosis characterized by the presence of occasional large lymphoid cells or immunoblasts in a polymorphous background. Necrosis is more commonly seen as compared to grade I lymphomatoid granulomatosis. By in situ hybridization, EBV-positive cells are readily seen. Uncertain Malignant Potential C38937 Grade II Meningioma Grade 2 Meningioma || WHO Grade II Meningioma An atypical meningioma which may recur in approximately 29-40% of the cases. This category includes the atypical meningioma, chordoid meningioma, and clear cell meningioma. Undetermined C7933 Grade III Lymphomatoid Granulomatosis Angiocentric Immunoproliferative Lesion Grade III || Grade III Angiocentric Immunoproliferative Lesion || Grade III Angiocentric Immunoproliferative Lesions Lymphomatoid granulomatosis characterized by the presence of aggregates of neoplastic large B-lymphocytes, usually admixed with pleomorphic and Hodgkin-like cells, in a background of chronic inflammation. Necrotic changes are present and are usually extensive. Grade III lymphomatoid granulomatosis should be approached clinically as a subtype of diffuse large B-cell lymphoma. Malignant C38938 Grade III Meningioma Grade 3 Meningioma || WHO Grade III Meningioma A malignant meningioma with aggressive clinical course. It recurs in approximately 50-78% of the cases. This category includes the anaplastic (malignant) meningioma, papillary meningioma, and rhabdoid meningioma. Malignant C3474 Granular Cell Tumor An unusual benign or malignant neoplasm characterized by the presence of neoplastic large polygonal cells with granular, eosinophilic cytoplasm which contains abundant lysosomes. It was originally thought to be a tumor originating from muscle cells and was named granular cell myoblastoma. Subsequent studies have suggested a derivation from Schwann cells. It affects females more often than males and it usually presents as a solitary mass. A minority of patients have multiple tumors. It can arise from many anatomic sites including the posterior pituitary gland, skin, oral cavity, esophagus, stomach, heart, mediastinum, and breast. Undetermined C35815 Granulocytic Sarcoma A tumor mass composed of myeloblasts, neutrophils and neutrophil precursors. Granulocytic sarcoma is the most common type of myeloid sarcoma. (WHO, 2001) Malignant C5963 Growth Hormone-Producing Pituitary Gland Carcinoma Growth Hormone Producing Pituitary Gland Carcinoma || Malignant Growth Hormone Producing Neoplasm of Pituitary || Malignant Growth Hormone Producing Neoplasm of Pituitary Gland || Malignant Growth Hormone Producing Neoplasm of the Pituitary || Malignant Growth Hormone Producing Neoplasm of the Pituitary Gland || Malignant Growth Hormone Producing Pituitary Gland Neoplasm || Malignant Growth Hormone Producing Pituitary Gland Tumor || Malignant Growth Hormone Producing Pituitary Neoplasm || Malignant Growth Hormone Producing Pituitary Tumor || Malignant Growth Hormone Producing Tumor || Malignant Growth Hormone Producing Tumor of Pituitary || Malignant Growth Hormone Producing Tumor of Pituitary Gland || Malignant Growth Hormone Producing Tumor of the Pituitary || Malignant Growth Hormone Producing Tumor of the Pituitary Gland || Malignant Growth Hormone Secreting Neoplasm of Pituitary || Malignant Growth Hormone Secreting Neoplasm of Pituitary Gland || Malignant Growth Hormone Secreting Neoplasm of the Pituitary || Malignant Growth Hormone Secreting Neoplasm of the Pituitary Gland || Malignant Growth Hormone Secreting Pituitary Gland Neoplasm || Malignant Growth Hormone Secreting Pituitary Gland Tumor || Malignant Growth Hormone Secreting Pituitary Neoplasm || Malignant Growth Hormone Secreting Pituitary Tumor || Malignant Growth Hormone Secreting Tumor of Pituitary || Malignant Growth Hormone Secreting Tumor of Pituitary Gland || Malignant Growth Hormone Secreting Tumor of the Pituitary || Malignant Growth Hormone Secreting Tumor of the Pituitary Gland || Malignant Pituitary Gland Somatotrophinoma || Malignant Pituitary Somatotrophinoma || Malignant Somatotrophinoma of Pituitary || Malignant Somatotrophinoma of Pituitary Gland || Malignant Somatotrophinoma of the Pituitary Gland A rare, hormonally functioning or non-functioning pituitary gland adenocarcinoma that produces growth hormone. It may be associated with acromegaly. Malignant C7402 Hairy Cell Leukemia Leukemic Reticuloendotheliosis A neoplasm of small B-lymphocytes with "hairy" projections in bone marrow, spleen, and peripheral blood. Most patients present with splenomegaly and pancytopenia. (WHO, 2001) Malignant C7401 Hairy Cell Leukemia Variant Prolymphocytic Variant of Hairy Cell Leukemia An indolent chronic B-cell leukemia resembling classic hairy cell leukemia but shows variant cytologic, hematologic, and immunophenotypic features and is resistant to the conventional therapy applied to hairy cell leukemia. Biologically, it is not related to hairy cell leukemia. Malignant C7602 Halo Nevus Leukoderma Acquisitum Centrifugum || Perinevoid Leukoderma || Perinevoid Vitiligo || Sutton Nevus A melanocytic nevus characterized by circumferential depigmentation. It is usually associated with a brisk lymphocytic infiltrate. Benign C6920 Hand-Schuller-Christian Disease A multifocal, unisystem form of Langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions. Undetermined C35850 Head and Neck Carcinoma Carcinoma of Head and Neck || Carcinoma of the Head and Neck A carcinoma that arises from the head and neck region. Representative examples include oral cavity squamous cell carcinoma, laryngeal squamous cell carcinoma, and salivary gland carcinoma. Malignant C34447 Head and Neck Squamous Cell Carcinoma Squamous Cell Carcinoma of Head and Neck || Squamous Cell Carcinoma of the Head and Neck A squamous cell carcinoma that arises from any of the following anatomic sites: lip and oral cavity, nasal cavity, paranasal sinuses, pharynx, larynx, and salivary glands. Malignant C3082 Heavy Chain Disease A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders. Malignant C27763 Helicobacter Pylori-Associated Gastric Mucosa-Associated Lymphoid Tissue Lymphoma Helicobacter Pylori-Associated Gastric MALT A low grade, indolent B-cell lymphoma that is associated with Helicobacter pylori infection. Malignant C27765 Helicobacter Pylori-Related Gastric Adenocarcinoma An adenocarcinoma that arises from the gastric mucosa and is caused by persistent infection with Helicobacter pylori. Malignant C3801 Hemangioblastoma Angioblastoma || Capillary Hemangioblastoma A WHO grade I tumor of uncertain histogenesis which is composed of stromal cells and abundant capillaries. Approximately 25% of hemangioblastomas are associated with Von Hippel-Lindau disease, a disease inherited through an autosomal dominant trait and characterized by the development of hemangioblastomas of the central nervous system and retina, clear cell renal carcinoma, pheochromocytoma, and pancreatic and inner ear tumors. (Adapted from WHO) Undetermined C3084 Hemangioendothelioma Angioendothelioma A vascular proliferation characterized by the presence of prominent endothelial cells and the formation of vascular channels. Hemangioendotheliomas may display borderline or low grade characteristics. Undetermined C3085 Hemangioma Angioma || Benign Angioma || Benign Hemangioma A benign vascular lesion characterized by the formation of capillary-sized or cavernous vascular channels. Benign C27686 Hepatitis Virus-Related Hepatocellular Carcinoma Hepatitis Virus Related Hepatocellular Carcinoma A hepatocellular carcinoma that develops following hepatitis virus exposure and injury of the liver parenchyma. Malignant C3728 Hepatoblastoma Pediatric Embryonal Hepatoma || Pediatric Hepatoblastoma A malignant liver neoplasm that occurs almost exclusively in infants, although isolated cases in older children and adults have been reported. Grossly, hepatoblastoma is solid, well circumscribed, and more often solitary than multiple. Microscopically, most of the tumors are composed exclusively of immature hepatocytic elements. About a fourth of hepatoblastomas contain a stromal component that may be undifferentiated or develop into bone or cartilage. The treatment of choice for hepatoblastoma is surgical excision with adjuvant therapy. Liver transplantation is being increasingly used as well. Malignant C3758 Hepatocellular Adenoma Adenoma of Liver Cells || Adenoma of the Liver Cells || Liver Cell Adenoma A benign epithelial neoplasm arising from the hepatocytes. Grossly, it appears as a soft, round mass which often contains areas of hemorrhage and necrosis. Morphologically, the neoplastic cells resemble normal hepatocytes and form plates separated by sinusoids. Most patients have a history of contraceptive or anabolic steroids use. Benign C3099 Hepatocellular Carcinoma Carcinoma of Liver Cells || Carcinoma of the Liver Cells || Hepatoma || Liver Cell Cancer (Hepatocellular Carcinoma) || Liver Cell Carcinoma || Primary Carcinoma of Liver Cells || Primary Carcinoma of the Liver Cells A malignant tumor that arises from hepatocytes. Hepatocellular carcinoma is relatively rare in the United States but very common in all African countries south of the Sahara and in Southeast Asia. Most cases are seen in patients over the age of 50 years, but this tumor can also occur in younger individuals and even in children. Hepatocellular carcinoma is more common in males than females and is associated with hepatitis B, hepatitis C, chronic alcohol abuse and cirrhosis. Serum elevation of alpha-fetoprotein occurs in a large percentage of patients with hepatocellular carcinoma. Grossly, hepatocellular carcinoma may present as a single mass, as multiple nodules, or as diffuse liver involvement. Microscopically, there is a wide range of differentiation from tumor to tumor (well differentiated to poorly differentiated tumors). Hepatocellular carcinomas quickly metastasize to regional lymph nodes and lung. The overall median survival of untreated liver cell carcinoma is about 4 months. The most effective treatment of hepatocellular carcinoma is complete resection of the tumor. Lately, an increasing number of tumors have been treated with liver transplantation. Malignant C8459 Hepatosplenic T-Cell Lymphoma Hepatosplenic Gamma/Delta T-Cell Lymphoma An extranodal, mature T-cell non-Hodgkin lymphoma that originates from cytotoxic T-cells, usually of gamma/delta T-cell type. It is characterized by the presence of medium-size neoplastic lymphocytes infiltrating the hepatic sinusoids. A similar infiltrating pattern is also present in the spleen and bone marrow that are usually involved at the time of the diagnosis. Malignant C53556 HER2 Positive Breast Carcinoma ERBB2 Overexpressing Subtype of Breast Carcinoma || HER2 Overexpressing Breast Carcinoma || HER2 Overexpressing Subtype of Breast Carcinoma || HER2 Positive Breast Cancer A biologic subset of breast carcinoma defined by high expression of HER2, GRB7, and TRAP100, and by lack of expression of estrogen receptor (ER). Malignant C4503 Hereditary Breast Carcinoma Familial Breast Carcinoma || Familial Cancer of Breast || Familial Cancer of the Breast || Hereditary Breast Cancer Breast carcinoma that has developed in relatives of patients with history of breast carcinoma. Malignant C36260 Hereditary Clear Cell Renal Cell Carcinoma Hereditary Conventional (Clear Cell) Renal Cell Carcinoma || Von Hippel Lindau-Deficient Clear Cell Renal Cell Carcinoma Hereditary clear cell renal cell carcinoma associated with biallelic loss/inactivation of the von Hippel-Lindau tumor suppressor gene. Malignant C40455 Hereditary Fallopian Tube Carcinoma Familial Fallopian Tube Carcinoma Fallopian tube carcinoma that has developed in relatives of patients that have a history of fallopian tube carcinoma. Malignant C43295 Hereditary Gastric Diffuse Adenocarcinoma Hereditary Diffuse Gastric Adenocarcinoma || Hereditary Diffuse Gastric Cancer An autosomal dominant inherited adenocarcinoma that arises from the gastric mucosa and is characterized by the presence of poorly cohesive malignant cells and absence of glandular formations. Malignant C8496 Hereditary Kidney Wilms Tumor Familial Kidney Wilms Tumor || Hereditary Kidney Adenosarcoma || Hereditary Kidney Nephroblastoma || Hereditary Renal Adenosarcoma Familial embryonal neoplasm derived from nephrogenic blastemal cells. Several lines of differentiation, including blastemal, stromal and epithelial, are usually expressed. Comprises approximately 1% of Wilms tumors. (AFIP fascicle version 2.0) Malignant C36102 Hereditary Ovarian Carcinoma Familial Ovarian Carcinoma Ovarian carcinoma that has developed in relatives of patients that have a history of ovarian carcinoma. Malignant C43298 Hereditary Pancreatic Carcinoma Familial Pancreatic Cancer || Familial Pancreatic Carcinoma || Hereditary Pancreatic Cancer A carcinoma that arises from the pancreas in a patient with a family history of pancreatic cancer. In the minority of cases, patients have recognized genetic syndromes (e.g., FAMMM syndrome, BRCA2 syndrome, Peutz-Jeghers syndrome) however, in the majority of cases the genetic cause has not been identified. Malignant C9222 Hereditary Papillary Renal Cell Carcinoma Familial Renal Papillary Carcinoma || Hereditary Kidney Papillary Carcinoma || Hereditary Papillary Carcinoma of Kidney || Hereditary Papillary Carcinoma of the Kidney || Hereditary Papillary Renal Carcinoma A familial carcinoma inherited in an autosomal dominant trait. It is characterized by the development of multiple, bilateral papillary renal cell carcinomas. The carcinomas range from microscopic lesions to clinically symptomatic tumors. It is associated with activating mutations of the MET oncogene. Malignant C103817 Hereditary Prostate Carcinoma Prostate carcinoma that has developed in relatives of patients with a history of prostate carcinoma. Malignant C8495 Hereditary Retinoblastoma Familial Retinoblastoma An inherited malignant tumor that originates in the nuclear layer of the retina. A predisposition to retinoblastoma has been associated with 13q14 cytogenetic abnormalities affecting the RB1 gene. Patients with the inherited form appear to be at increased risk for secondary non-ocular malignancies such as osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. Malignant C46099 Hereditary Thyroid Gland Medullary Carcinoma Familial Thyroid Gland Medullary Carcinoma A medullary thyroid gland carcinoma inherited through an autosomal dominant mode. It is associated with multiple endocrine neoplasia type II (2a) or III (2b). Malignant C54664 Hidradenocarcinoma Clear Cell Eccrine Carcinoma A carcinoma with apocrine and less often eccrine differentiation, arising from the sweat glands. It usually presents as a solitary slow growing nodule in the dermis or subcutaneous tissues. It is characterized by a nodular growth pattern and it is often associated with necrotic changes. Malignant C7563 Hidradenoma Acrospiroma || Eccrine Acrospiroma A benign epithelial neoplasm arising from the sweat glands. Variants include the clear cell and nodular hidradenoma. Benign C3760 Hidrocystoma Hydrocystoma A benign cystic proliferation of the sweat glands with apocrine or eccrine differentiation. It usually presents as a dome-shaped, cystic papular or nodular lesion usually in the face and neck. It is a unilocular or mutlilocular lesion lined by an inner and an outer layer of epithelium. Complete excision is usually curative. Benign C102897 High Grade Astrocytic Tumor High Grade Astrocytic Neoplasm || High-Grade Astrocytic Neoplasm || High-Grade Astrocytic Tumor An anaplastic astrocytoma (grade III astrocytic tumor) or glioblastoma (grade IV astrocytic tumor). Malignant C125904 High Grade B-Cell Lymphoma with MYC and BCL2 or BCL6 Rearrangements DH Lymphoma || Double Hit Lymphoma || Double-Hit Lymphoma || High-Grade B-Cell Lymphoma with MYC and BCL2 or BCL6 Rearrangements A rare B-cell non-Hodgkin lymphoma that is characterized by the abnormal rearrangement of two genes, MYC gene and either BCL2 or BCL6 genes. Patients with this type of lymphoma usually respond poorly to standard treatments and have a poor prognosis. Malignant C80291 High Grade B-Cell Lymphoma, Not Otherwise Specified High Grade B-Cell Lymphoma, NOS || High-Grade B-Cell Lymphoma, NOS || High-Grade B-Cell Lymphoma, Not Otherwise Specified A high grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma which lacks MYC, BCL2, and BCL6 rearrangements. Malignant C105555 High Grade Ovarian Serous Adenocarcinoma High-Grade Ovarian Serous Adenocarcinoma A rapidly growing serous adenocarcinoma that arises from the ovary. It is characterized by the presence of high grade cytologic features and frequent mitotic figures. Malignant C36077 Hilar Cholangiocarcinoma Hilar CC || Klatskin Tumor || Perihilar Cholangiocarcinoma A carcinoma that arises from the junction, or adjacent to the junction, of the right and left hepatic ducts. Malignant C9294 Histiocytic and Dendritic Cell Neoplasm Histiocytic and Dendritic Cell Neoplasms || Histiocytic and Dendritic Cell Tumors Rare tumors that affect the hematopoietic and lymphoid tissues. The cells of origin are the histiocytes and accessory cells. They can occur at any age and show no significant variations in geographical distribution. This category includes the histiocytic sarcoma, Langerhans cell histiocytosis, Langerhans cell sarcoma, interdigitading dendritic cell sarcoma/tumor, follicular dendritic cell sarcoma/tumor, and dendritic cell sarcoma, not otherwise specified. (WHO, 2001) Undetermined C27349 Histiocytic Sarcoma An aggressive malignant neoplasm with a poor response to therapy, usually presenting as stage III/IV disease. It is characterized by the presence of neoplastic cells with morphologic and immunophenotypic characteristics similar to those seen in mature histiocytes. Malignant C9357 Hodgkin Lymphoma Hodgkin's Disease || Hodgkin's Lymphoma A lymphoma, previously known as Hodgkin's disease, characterized by the presence of large tumor cells in an abundant admixture of nonneoplastic cells. There are two distinct subtypes: nodular lymphocyte predominant Hodgkin lymphoma and classical Hodgkin lymphoma. Hodgkin lymphoma involves primarily lymph nodes. Malignant C27684 Human Papillomavirus-Related Adenocarcinoma HPV-Related Adenocarcinoma || Human Papilloma Virus Related Adenocarcinoma || Human Papilloma Virus-Related Adenocarcinoma An adenocarcinoma associated with the presence of human papillomavirus infection. Malignant C27676 Human Papillomavirus-Related Cervical Squamous Cell Carcinoma HPV-Associated Cervical Squamous Cell Carcinoma || HPV-Related Cervical Squamous Cell Carcinoma || HPVA Cervical Squamous Cell Carcinoma || Human Papilloma Virus Related Cervical Squamous Cell Carcinoma || Human Papilloma Virus-Related Cervical Squamous Cell Carcinoma || Human Papillomavirus-Associated Cervical Squamous Cell Carcinoma A cervical squamous cell carcinoma associated with human papillomavirus infection. Malignant C27679 Human Papillomavirus-Related Vulvar Squamous Cell Carcinoma HPV-Related Vulvar Squamous Cell Carcinoma || Human Papilloma Virus Related Vulvar Squamous Cell Carcinoma || Human Papilloma Virus-Related Vulvar Squamous Cell Carcinoma A squamous cell carcinoma that arises from the vulva and is caused by human papillomavirus infection. Malignant C121686 Hybrid Nerve Sheath Tumor Hybrid Nerve Sheath Neoplasm || Hybrid Neurofibroma-Perineurioma || Hybrid Neurofibroma-Schwannoma || Hybrid Schwannoma-Perineurioma || Nerve Sheath Tumor, NOS || Nerve Sheath Tumor, Not Otherwise Specified A benign nerve sheath tumor characterized by the combination of histologic features seen in schwannomas, neurofibromas, and perineuriomas. Benign C45327 Hydroa Vacciniforme-Like Lymphoproliferative Disorder HV-Like LPD A rare, EBV-positive cutaneous T-cell lymphoproliferative disorder, composed of CD8 positive cytotoxic T-lymphocytes. It affects children, almost exclusively in Latin America and Asia. Patients present with papulovesicular skin lesions, clinically resembling hydroa vacciniforme, in areas of sun-exposed skin. Malignant C80344 Hyperdiploid B Acute Lymphoblastic Leukemia Hyperdiploid ALL || Hyperdiploid Acute Lymphoblastic Leukemia || Hyperdiploid B-Acute Lymphoblastic Leukemia || Hyperdiploid B-Cell Acute Lymphoblastic Leukemia A B-cell acute leukemia characterized by the presence of lymphoblasts which contain more than 50 and usually less than 66 chromosomes. It is commonly seen in children and rarely in adults. It has a favorable clinical outcome. Malignant C122686 Hypocellular Myelodysplastic Syndrome Myelodysplastic syndrome characterized by decreased cellularity in the bone marrow. Malignant C80345 Hypodiploid B Acute Lymphoblastic Leukemia Hypodiploid ALL || Hypodiploid Acute Lymphoblastic Leukemia || Hypodiploid B-Acute Lymphoblastic Leukemia || Hypodiploid B-Cell Acute Lymphoblastic Leukemia A B-cell acute leukemia characterized by the presence of lymphoblasts which contain less than 46 chromosomes. It occurs in both children and adults. It has an unfavorable clinical outcome. Malignant C4043 Hypopharyngeal Squamous Cell Carcinoma Epidermoid Carcinoma of Hypopharynx || Epidermoid Carcinoma of the Hypopharynx || Hypopharyngeal Conventional Squamous Cell Carcinoma || Hypopharyngeal Epidermoid Carcinoma || Squamous Cell Carcinoma of Hypopharynx || Squamous Cell Carcinoma of the Hypopharynx A squamous cell carcinoma arising from the hypopharynx. Signs and symptoms include dysphagia, hemoptysis, and the presence of a neck mass. Malignant C4286 Immature Teratoma Grade 2 Teratoma A teratoma characterized by the presence of an extensive component of immature, fetal-type tissues. Undetermined C3461 Immunoblastic Lymphoma A diffuse large B-cell lymphoma characterized by the presence of immunoblasts with uniformly round-to-oval nuclei, a prominent nucleolus, and abundant cytoplasm. Malignant C81767 Indeterminate Dendritic Cell Tumor Indeterminate Cell Histiocytosis A very rare dendritic cell tumor composed of spindle to ovoid cells with a phenotype that is similar to the Langerhans cells. Patients usually present with cutaneous papules, nodules, and plaques. Systemic symptoms are usually absent. The clinical course is variable. Undetermined C122617 Infant Acute Lymphoblastic Leukemia with MLL Rearrangement An acute lymphoblastic leukemia with rearrangement of the MLL (KMT2A) gene that occurs in infancy. Malignant C122621 Infant Acute Lymphoblastic Leukemia without MLL Gene Rearrangement Infant Acute Lymphoblastic Leukemia without KMT2A Gene Rearrangement An acute lymphoblastic leukemia without rearrangement of the MLL (KMT2A) gene that occurs in infancy. Malignant C122603 Infant Leukemia Leukemia in Infancy An acute lymphoblastic or acute myeloid leukemia that occurs in infancy. Malignant C4244 Infantile Fibrosarcoma Congenital Fibrosarcoma A fibrosarcoma that occurs in infants. It shares identical morphologic features with adult fibrosarcoma but carries the t(12;15)(p13;q25) translocation that results in ETV6-NTRK3 gene fusion. It usually affects the superficial and deep soft tissues of the extremities. The prognosis is generally much more favorable than for adult fibrosarcoma, and it rarely metastasizes. Malignant C27885 Infiltrating Bladder Urothelial Carcinoma Infiltrating Transitional Cell Carcinoma of the Urinary Bladder || Invasive Bladder Transitional Cell Carcinoma || Invasive Bladder Urothelial Carcinoma || Invasive Transitional Cell Carcinoma of the Urinary Bladder An invasive transitional cell carcinoma that arises from the urinary bladder urothelium. Malignant C8577 Infiltrating Cervical Carcinoma A carcinoma that arises from the cervix and invades into the stromal tissue. Malignant C6186 Infiltrating Renal Pelvis Urothelial Carcinoma, Sarcomatoid Variant Kidney Pelvis Sarcomatoid Transitional Cell Carcinoma || Sarcomatoid Transitional Cell Carcinoma of Kidney Pelvis || Sarcomatoid Transitional Cell Carcinoma of Renal Pelvis || Sarcomatoid Transitional Cell Carcinoma of the Kidney Pelvis || Sarcomatoid Transitional Cell Carcinoma of the Renal Pelvis An infiltrating transitional cell carcinoma that arises from the renal pelvis and exhibits sarcomatoid features. Malignant C27495 Inflammatory Leiomyosarcoma A morphologic variant of leiomyosarcoma characterized by the presence of an inflammatory infiltrate admixed with malignant spindle cells. Malignant C6481 Inflammatory Myofibroblastic Tumor Inflammatory Myofibroblastic Neoplasm A multinodular intermediate fibroblastic neoplasm that arises from soft tissue or viscera, in children and young adults. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes, and plasma cells. Undetermined C9282 Interdigitating Dendritic Cell Sarcoma Interdigitating Cell Sarcoma/Tumor || Interdigitating Dendritic Cell Sarcoma/Tumor A neoplastic proliferation of spindle to ovoid cells which show phenotypic features similar to those of interdigitating dendritic cells. The clinical course is generally aggressive. (WHO, 2008) Malignant C80298 Intestinal Follicular Lymphoma Primary Intestinal Follicular Lymphoma A follicular lymphoma arising from the intestine. The majority of cases occur in the small intestine, particularly the duodenum. Usually the patients have localized disease and a favorable clinical outcome. Malignant C4126 Intestinal-Type Adenocarcinoma Intestinal Type Carcinoma An adenocarcinoma arising from epithelium which has undergone intestinal metaplasia. Representative examples include gastric, gallbladder, and ampulla of Vater intestinal type adenocarcinomas. Malignant C96947 Intrahepatic Bile Duct Mucinous Cystic Neoplasm with an Associated Invasive Carcinoma Intrahepatic Bile Duct Cystadenocarcinoma || Intrahepatic Bile Duct MCN with an Associated Invasive Carcinoma A mucinous cystic neoplasm that arises from the intrahepatic bile ducts and it is associated with an invasive carcinomatous component. Malignant C35417 Intrahepatic Cholangiocarcinoma Intrahepatic Bile Duct Carcinoma || Intrahepatic Carcinoma of Bile Duct || Intrahepatic Carcinoma of the Bile Duct A carcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. Grossly, the malignant lesions are solid, nodular, and grayish. Morphologically, the vast majority of cases are adenocarcinomas. Signs and symptoms include malaise, weight loss, right upper quadrant abdominal pain, and night sweats. Early detection is difficult and the prognosis is generally poor. Malignant C95397 Intramucosal Adenocarcinoma A neoplastic lesion that shows morphologic evidence of invasion into the lamina propria or muscularis mucosa. There is no evidence of invasion into the submucosa. Evidence of invasion may refer to stromal invasion by single cells or clusters of cells, presence of atypical and complex glandular architectural patterns beyond those that are present in normal mucosa, desmoplasia, and/or vascular invasion. Malignant C7846 Intraocular Retinoblastoma Retinoblastoma restricted to local involvement. Malignant C4342 Intravascular Large B-Cell Lymphoma Angiotropic Large Cell Lymphoma || Angiotropic Lymphoma || Intravascular B-Cell Lymphoma || Malignant Angioendotheliomatosis A rare extranodal B-cell non-Hodgkin lymphoma, characterized by the presence of lymphoma cells exclusively in the lumina of small vessels, particularly capillaries. This is an extremely aggressive lymphoma which responds poorly to chemotherapy. (WHO, 2001) Malignant C4194 Invasive Breast Carcinoma of No Special Type Infiltrating Ductal Adenocarcinoma || Infiltrating Ductal Breast Carcinoma || Infiltrating Ductal Carcinoma || Infiltrating Ductal Carcinoma of Breast || Infiltrating Ductal Carcinoma of the Breast || Invasive Ductal Adenocarcinoma || Invasive Ductal Breast Carcinoma || Invasive Ductal Carcinoma || Invasive Ductal Carcinoma of Breast || Invasive Ductal Carcinoma of the Breast || Invasive Ductal Carcinoma, NOS || Invasive Ductal Carcinoma, NST || Invasive Ductal Carcinoma, No Specific Type || Invasive Ductal Carcinoma, Not Otherwise Specified A term that refers to a large and heterogeneous group of invasive breast carcinomas that cannot be classified morphologically as any of the special histological types. (WHO 2019) Malignant C7950 Invasive Breast Lobular Carcinoma Classic Invasive Lobular Carcinoma || Infiltrating Lobular Adenocarcinoma || Infiltrating Lobular Breast Carcinoma || Infiltrating Lobular Carcinoma of Breast || Infiltrating Lobular Carcinoma of the Breast || Invasive Lobular Adenocarcinoma || Invasive Lobular Breast Carcinoma || Invasive Lobular Carcinoma || Invasive Lobular Carcinoma of Breast || Invasive Lobular Carcinoma of the Breast || Invasive Lobular Carcinoma, Classic Type An infiltrating lobular adenocarcinoma of the breast. The malignant cells lack cohesion and are arranged individually or in a linear manner (Indian files), or as narrow trabeculae within the stroma. The malignant cells are usually smaller than those of ductal carcinoma, are less pleomorphic, and have fewer mitotic figures. Malignant C36085 Invasive Breast Papillary Carcinoma Infiltrating Papillary Breast Carcinoma || Invasive Papillary Breast Carcinoma An invasive breast carcinoma characterized by the presence of papillary structures. Malignant C9480 Invasive Carcinoma A carcinoma that is not confined to the epithelium, and has spread to the surrounding stroma. Malignant C7904 Invasive Malignant Thymoma Malignant Thymoma, Invasive || Thymoma Malignant Invasive A malignant thymoma that extends beyond the capsule and infiltrates the surrounding tissues. Malignant C4231 Junctional Nevus Intraepidermal Nevus || Intraepidermal Nevus of Skin || Intraepidermal Nevus of the Skin || Junction Nevus || Junctional Melanocytic Nevus || Junctional Melanocytoma || Junctional Nevus of Skin || Junctional Nevus of the Skin || Junctional Skin Nevus A nevus characterized by the presence of an intraepidermal proliferation of nevus cells. The nevus cells form multiple nests in the dermal-epidermal junction. It presents as a small, slightly raised, pigmented skin lesion. Benign C9233 Juvenile Myelomonocytic Leukemia A myelodysplastic/myeloproliferative neoplasm of childhood that is characterized by proliferation principally of the granulocytic and monocytic lineages. Myelomonocytic proliferation is seen in the bone marrow and the blood. The leukemic cells may infiltrate any tissue, however liver, spleen, lymph nodes, skin, and respiratory tract are the most common sites of involvement. (WHO, 2001) Malignant C9087 Kaposi Sarcoma Kaposi's Sarcoma || Multiple Hemorrhagic Sarcoma A malignant neoplasm characterized by a vascular proliferation which usually contains blunt endothelial cells. Erythrocyte extravasation and hemosiderin deposition are frequently present. The most frequent site of involvement is the skin; however it may also occur internally. It generally develops in people with compromised immune systems including those with acquired immune deficiency syndrome (AIDS). Malignant C27510 Kaposiform Hemangioendothelioma An intermediate, locally aggressive blood vessel neoplasm characterized by the presence of spindle endothelial cells, and formation of capillary-sized and slit-like vascular channels. It is often associated with hemorrhage and hemosiderin deposition. Undetermined C3146 Keratoacanthoma Cutaneous Keratoacanthoma || Skin Keratoacanthoma A dome-shaped, rapidly growing skin lesion composed of well differentiated squamous cells. It usually occurs on sun-exposed areas of the body, most often the head, neck, and limbs. It is considered a variant of well-differentiated squamous cell carcinoma with distinct clinical behavior. Keratoacanthomas affect males more frequently than females and the majority tend to regress spontaneously. Malignant C9384 Kidney Carcinoma Kidney Cancer || Renal Cancer || Renal Carcinoma A carcinoma arising from the epithelium of the renal parenchyma or the renal pelvis. The majority are renal cell carcinomas. Kidney carcinomas usually affect middle aged and elderly adults. Hematuria, abdominal pain, and a palpable mass are common symptoms. Malignant C7572 Kidney Medullary Carcinoma Renal Medullary Carcinoma A type of renal carcinoma affecting mostly young African-Americans. It is located in the medulla of the kidney, and follows an aggressive clinical course. Most reported cases have shown metastatic disease at the time of diagnosis. Malignant C4526 Kidney Oncocytoma Oncocytoma of Kidney || Oncocytoma of the Kidney || Renal Epithelial Oncocytic Neoplasm || Renal Epithelial Oncocytic Tumor || Renal Oncocytoma A benign tumor of the kidney, characterized by the presence of large cells with abundant eosinophilic granular cytoplasm. The majority of these tumors are discovered incidentally, during work-up of other conditions. Benign C40407 Kidney Wilms Tumor Kidney Nephroblastoma || Renal Wilms Tumor || Renal Wilms' Tumor || Wilms Tumor of the Kidney || Wilms' Tumor of the Kidney An embryonal pediatric tumor of the kidney which may also be seen rarely in adults. The peak incidence of Wilms tumor is between the second and fifth year of life. Microscopically, it is composed of a mixture of cellular elements (blastemal, stromal, and epithelial). The most common sites of metastasis include the regional lymph nodes, lungs, and liver. Malignant C9363 Labia Majora Carcinoma Carcinoma of Labia Majora || Carcinoma of the Labia Majora || Labia Majora Cancer A carcinoma that arises from the labia majora. Malignant C9364 Labia Minora Carcinoma Carcinoma of Labia Minora || Carcinoma of the Labia Minora || Labia Minora Cancer A carcinoma that arises from the labia minora. Malignant C6129 Lacrimal Gland Carcinoma Carcinoma of Lacrimal Gland || Carcinoma of the Lacrimal Gland A carcinoma that arises from the lacrimal glands. Representative examples include adenocarcinoma, carcinoma ex pleomorphic adenoma, squamous cell carcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma. Malignant C6804 Lacrimal Gland Carcinoma ex Pleomorphic Adenoma Carcinoma ex Pleomorphic Adenoma of Lacrimal Gland || Carcinoma ex Pleomorphic Adenoma of the Lacrimal Gland || Lacrimal Gland Malignant Mixed Neoplasm || Lacrimal Gland Malignant Mixed Tumor || Malignant Mixed Neoplasm of Lacrimal Gland || Malignant Mixed Neoplasm of the Lacrimal Gland || Malignant Mixed Tumor of Lacrimal Gland || Malignant Mixed Tumor of the Lacrimal Gland A carcinoma arising in a pre-existing pleomorphic adenoma in the lacrimal gland. Malignant C4542 Lacrimal Gland Pleomorphic Adenoma Benign Mixed Tumor of Lacrimal Gland || Benign Mixed Tumor of the Lacrimal Gland || Lacrimal Gland Benign Mixed Tumor || Pleomorphic Adenoma of Lacrimal Gland || Pleomorphic Adenoma of the Lacrimal Gland A benign, usually encapsulated neoplasm of the lacrimal gland composed of epithelial and mesenchymal cells. Pleomorphic adenomas are neoplasms that develop in the salivary glands or heterotopic salivary gland tissues. It has been suggested that myoepithelial cells play a major role in the histogenesis of these tumors. In the lacrimal gland, pleomorphic adenomas presumably develop from metaplastic myoepithelial cells. Benign C9473 Lactating Adenoma Pregnancy Adenoma A tubular type adenoma of the breast in which, during pregnancy and lactation, the epithelial cells show extensive secretory changes. Benign C3342 Lactotroph Adenoma Lactotrope Adenoma || Lactotroph Cell Adenoma || PRL Producing Pituitary Gland Adenoma || Pituitary Gland Prolactinoma || Pituitary Prolactinoma || Prolactin Producing Adenoma of Pituitary || Prolactin Producing Adenoma of Pituitary Gland || Prolactin Producing Adenoma of the Pituitary || Prolactin Producing Adenoma of the Pituitary Gland || Prolactin Producing Pituitary Adenoma || Prolactin Producing Pituitary Gland Adenoma || Prolactin Secreting Adenoma || Prolactin Secreting Adenoma of Pituitary || Prolactin Secreting Adenoma of Pituitary Gland || Prolactin Secreting Adenoma of the Pituitary || Prolactin Secreting Adenoma of the Pituitary Gland || Prolactin Secreting Pituitary Adenoma || Prolactin Secreting Pituitary Gland Adenoma || Prolactin-Producing Pituitary Gland Adenoma || Prolactinoma || Prolactinoma of Pituitary || Prolactinoma of Pituitary Gland || Prolactinoma of the Pituitary || Prolactinoma of the Pituitary Gland An adenoma of the anterior lobe of the pituitary gland that produces prolactin. It is the most common type of pituitary gland adenomas and it is associated with hyperprolactinemia. Clinical manifestations include amenorrhea, galactorrhea, impotence, headache, and visual disturbances. Benign C3107 Langerhans Cell Histiocytosis Langerhans Cell Granulomatosis || Langerhans Cell Histiocytosis, NOS || Langerhans Cell Histiocytosis, Not Otherwise Specified A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) Undetermined C6921 Langerhans Cell Sarcoma A neoplastic proliferation of Langerhans cells with overtly malignant cytologic features. It can be considered a higher grade variant of Langerhans cell histiocytosis (LCH) and it can present de novo or progress from antecedent LCH. (WHO, 2001) Malignant C3780 Large Cell Carcinoma Carcinoma, Large Cell A malignant epithelial neoplasm composed of large, atypical cells. Malignant C6904 Large Cell Medulloblastoma A medulloblastoma composed of large cells with prominent nucleoli and a larger amount of cytoplasm in contrast with the cells of the classic medulloblastoma. Malignant C6875 Large Cell Neuroendocrine Carcinoma Large Cell NEC || Large-cell neuroendocrine carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation. It is composed of malignant large cells. Malignant C129436 Large Cell/Anaplastic Medulloblastoma A medulloblastoma composed of sheets of large cells mixed with cells characterized by marked nuclear pleomorphism and high mitotic activity. Malignant C4044 Laryngeal Squamous Cell Carcinoma Epidermoid Carcinoma of Larynx || Epidermoid Carcinoma of the Larynx || Laryngeal Conventional Squamous Cell Carcinoma || Laryngeal Epidermoid Carcinoma || Larynx Epidermoid Carcinoma || Larynx Squamous Cell Carcinoma || Squamous Cell Carcinoma of Larynx || Squamous Cell Carcinoma of the Larynx A squamous cell carcinoma that arises from the larynx. It is the most common histologic type of laryngeal carcinoma. It can arise from the glottis, supraglottic area, or it can be transglottic. Glottic squamous cell carcinoma is the most frequent laryngeal carcinoma in the United States. The symptoms, clinical behavior and the prognosis depend on the site of origin within the larynx. Malignant C3157 Leiomyoma Fibroid || Leiomyomatous Tumor A well-circumscribed benign smooth muscle neoplasm characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern. Benign C3158 Leiomyosarcoma An uncommon, aggressive malignant smooth muscle neoplasm, usually occurring in post-menopausal women. It is characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid, granular cell, inflammatory and myxoid leimyosarcomas. Malignant C9151 Lentigo Maligna Melanoma Malignant Lentigo Melanoma || Melanoma in Hutchinson Melanotic Freckle A melanoma of the skin characterized by single cell infiltration of the papillary dermis by atypical melanocytes, in a background of lentigo maligna changes. Malignant C123160 Lepidic Predominant Adenocarcinoma Lepidic Predominant Lung Adenocarcinoma A lung adenocarcinoma characterized by the presence of mildly and moderately differentiated adenocarcinoma cells across the alveolar walls with at least one focus of invasive carcinoma measuring more than 5 mm in greatest dimension. Malignant C3160 Letterer-Siwe Disease A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia. Malignant C3161 Leukemia Blood (Leukemia) A malignant (clonal) hematologic disorder, involving hematopoietic stem cells and characterized by the presence of primitive or atypical myeloid or lymphoid cells in the bone marrow and the blood. Leukemias are classified as acute or chronic based on the degree of cellular differentiation and the predominant cell type present. Leukemia is usually associated with anemia, fever, hemorrhagic episodes, and splenomegaly. Common leukemias include acute myeloid leukemia, chronic myelogenous leukemia, acute lymphoblastic or precursor lymphoblastic leukemia, and chronic lymphocytic leukemia. Treatment is vital to patient survival; untreated, the natural course of acute leukemias is normally measured in weeks or months, while that of chronic leukemias is more often measured in months or years. Malignant C121153 LH-Producing Gonadotroph Adenoma LH-Producing Pituitary Gland Adenoma || Luteinizing Hormone-Producing Gonadotroph Adenoma An adenoma of the anterior lobe of the pituitary gland that produces luteinizing hormone (LH). Benign C9315 Lip and Oral Cavity Carcinoma Lip and Oral Cavity Cancer || Oral Cancer || Oral Carcinoma A carcinoma arising in the lip or oral cavity. Most oral cavity carcinomas are squamous cell carcinomas of the tongue, buccal mucosa, or gums. Less frequent morphologic variants include mucoepidermoid carcinoma and adenocarcinoma. Lip carcinomas are usually basal cell or squamous cell carcinomas. Malignant C42690 Lip and Oral Cavity Squamous Cell Carcinoma A squamous cell carcinoma arising from the lip or the oral cavity. The oral cavity squamous cell carcinoma usually arises from the buccal mucosa, tongue, or gums. It occurs predominantly in adults who use tobacco and alcohol and has a tendency to metastasize early to lymph nodes. Malignant C8014 Lip Basal Cell Carcinoma Basal Cell Carcinoma of Lip || Basal Cell Carcinoma of the Lip A basal cell carcinoma arising from the lip. Malignant C3490 Lip Carcinoma Carcinoma of Lip || Carcinoma of the Lip A malignant epithelial neoplasm arising from the lips. Malignant C4042 Lip Squamous Cell Carcinoma Lip SCC || SCC of Lip || SCC of the Lip || Squamous Cell Carcinoma of Lip || Squamous Cell Carcinoma of the Lip A squamous cell carcinoma that arises from the lip. It affects males more often than females and it usually involves the lower lip. Risk factors include UV exposure, alcohol consumption, smoking, and immunosuppression. Malignant C27483 Lipoblastoma Embryonic Lipoma || Fetal Lipoma || Infantile Lipoma A lipoma usually occurring in the extremities of young children (usually boys). It is characterized by lobules of adipose tissue, separated by fibrous septa. The adipose tissue is composed of mature adipocytes and lipoblasts. The lipoblasts may be scarce, depending on the age of the patient. Benign C3192 Lipoma A benign, usually painless, well-circumscribed lipomatous tumor composed of adipose tissue. Benign C3193 Lipomatosis Launois-Bensaude Syndrome || Madelung Disease || Madelung's Disease A neoplastic process characterized by diffuse overgrowth of mature adipose tissue. Benign C3194 Liposarcoma Liposarcoma Not Otherwise Specified || Malignant Lipomatous Neoplasm A usually painless malignant tumor that arises from adipose tissue. Microscopically, it may contain a spectrum of neoplastic adipocytes ranging from lipoblasts to pleomorphic malignant adipocytes. Morphologic variants include: well differentiated, dedifferentiated, pleomorphic, and myxoid liposarcoma. The metastatic potential is higher in less differentiated tumors. Malignant C7927 Liver and Intrahepatic Bile Duct Carcinoma Cancer of Liver || Cancer of Liver and Intrahepatic Biliary Tract || Cancer of the Liver || Cancer of the Liver and Intrahepatic Biliary Tract || Liver Cancer || Liver Carcinoma || Liver and Intrahepatic Bile Duct Cancer || Liver and Intrahepatic Biliary Tract Cancer || Liver and Intrahepatic Biliary Tract Carcinoma || Primary Liver Carcinoma A carcinoma that arises from the hepatocytes or intrahepatic bile ducts. The main subtypes are hepatocellular carcinoma (hepatoma) and cholangiocarcinoma. Malignant C96848 Liver Carcinosarcoma Hepatic Carcinosarcoma An aggressive carcinoma with a sarcomatous component that arises from the liver. The sarcomatous component is clonally related to the more differentiated, carcinomatous component. The prognosis is usually poor. Malignant C42598 Low Grade Appendix Mucinous Neoplasm Appendix Well Differentiated Mucinous Adenocarcinoma || Low Grade Appendiceal Mucinous Neoplasm || Low-Grade Appendiceal Mucinous Neoplasm || Low-Grade Appendix Mucinous Neoplasm A well differentiated mucinous neoplasm that arises from the appendix. It is characterized by slow growth and it is associated with the development of pseudomyxoma peritonei. Malignant C116342 Low Grade Astrocytoma Low Grade Astrocytic Neoplasm || Low Grade Astrocytic Tumor || Low-Grade Astrocytic Neoplasm || Low-Grade Astrocytic Tumor || Low-Grade Astrocytoma A grade I or grade II astrocytoma. This category includes pilocytic astrocytoma (grade I), subependymal giant cell astrocytoma (grade I), and diffuse astrocytoma (grade II). Undetermined C4263 Low Grade Endometrioid Stromal Sarcoma Endolymphatic Stromal Myosis An infiltrating mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. It is characterized by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. It is usually characterized by indolent growth and late recurrences. Malignant C105556 Low Grade Ovarian Serous Adenocarcinoma Invasive Ovarian Micropapillary Serous Carcinoma || Low-Grade Ovarian Serous Adenocarcinoma A slow-growing serous adenocarcinoma that arises from the ovary. It usually originates from borderline neoplastic processes or adenofibromas. It is characterized by the presence of low grade cytologic features and infrequent mitotic figures. Malignant C9152 Low-CSD Melanoma Cutaneous Superficial Spreading Melanoma || Low Degree of Cumulative Sun Damage Melanoma || Low-CSD Melanoma/SSM || Low-CSD Melanoma/Superficial Spreading Melanoma || Non- CSD Melanoma || Pagetoid Melanoma || Superficial Spreading Malignant Melanoma of Skin || Superficial Spreading Malignant Melanoma of the Skin || Superficial Spreading Malignant Skin Melanoma || Superficial Spreading Melanoma of Skin || Superficial Spreading Melanoma of the Skin A type of melanoma that typically occurs in light-skinned individuals ranging in age from young adults to the elderly. Risk factors include extensive sun exposure during childhood, a family history of melanoma, and the presence of dysplastic nevi. Malignant C8171 Lower Gingival Squamous Cell Carcinoma Lower Gingival SCC || Lower Gum SCC || Lower Gum Squamous Cell Carcinoma || SCC of Lower Gingiva || SCC of Lower Gum || SCC of the Lower Gingiva || SCC of the Lower Gum || Squamous Cell Carcinoma of Lower Gingiva || Squamous Cell Carcinoma of Lower Gum || Squamous Cell Carcinoma of the Lower Gingiva || Squamous Cell Carcinoma of the Lower Gum A squamous cell carcinoma of the oral cavity that arises from the lower gingiva. Malignant C53554 Luminal A Breast Carcinoma Luminal A || Luminal A Breast Cancer || Luminal A Estrogen Receptor Positive Subtype of Breast Carcinoma || Luminal A Subtype of Breast Carcinoma A biologic subset of breast carcinoma defined by high expression of genes characteristic of luminal epithelial cells, including estrogen receptor (ER), estrogen regulated protein LIV-1, and the transcription factors hepatocyte nuclear factor 3, HNF3A, XBP1, and GATA 3. This subtype of breast cancer is associated with a good prognosis. Malignant C53555 Luminal B Breast Carcinoma Luminal B || Luminal B Breast Cancer || Luminal B Estrogen Receptor Positive Subtype of Breast Carcinoma || Luminal B Subtype of Breast Carcinoma A biologic subset of breast carcinoma defined by low to moderate expression of genes characteristic of luminal epithelial cells including estrogen receptor (ER), and high expression of GGH, LAPTM4B, and CCNE1. This subtype of breast cancer is associated with a good prognosis, although not as favorable as the luminal A subtype. Malignant C5649 Lung Acinar Adenocarcinoma Acinar Adenocarcinoma of Lung || Acinar Adenocarcinoma of the Lung A morphologic variant of lung adenocarcinoma characterized by the presence of acinar structures composed of columnar or cuboidal cells. (NCI05) Malignant C3512 Lung Adenocarcinoma Adenocarcinoma of Lung || Adenocarcinoma of the Lung A carcinoma that arises from the lung and is characterized by the presence of malignant glandular epithelial cells. There is a male predilection with a male to female ratio of 2:1. Usually lung adenocarcinoma is asymptomatic and is identified through screening studies or as an incidental radiologic finding. If clinical symptoms are present they include shortness of breath, cough, hemoptysis, chest pain, and fever. Tobacco smoke is a known risk factor. Malignant C5666 Lung Adenoid Cystic Carcinoma Adenocystic Carcinoma of Lung || Adenocystic Carcinoma of the Lung || Adenoid Cystic Carcinoma of Lung || Adenoid Cystic Carcinoma of the Lung || Lung Adenocystic Carcinoma || Pulmonary Adenocystic Carcinoma || Pulmonary Adenoid Cystic Carcinoma A rare usually indolent lung carcinoma characterized by a cribiform and tubular pattern and the presence of glandular epithelial cells. Clinical symptoms include shortness of breath, cough, wheeze, hemopytsis and chest pain. Malignant C9133 Lung Adenosquamous Carcinoma Adenosquamous Cell Lung Carcinoma || Adenosquamous Lung Carcinoma An aggressive carcinoma with a poor prognosis characterized by a presence of both malignant squamous cells and glandular cells. Malignant C7266 Lung Basaloid Large Cell Carcinoma A large cell lung carcinoma characterized by the presence of a solid nodular or anastomotic trabecular growth pattern, peripheral palisading, and comedo type necrosis. Malignant C45507 Lung Basaloid Squamous Cell Carcinoma Basaloid Squamous Cell Lung Carcinoma || Squamous Cell Lung Carcinoma, Basaloid Variant A morphologic variant of squamous cell lung carcinoma characterized by nuclear palisading. Malignant C4878 Lung Carcinoma Cancer of Lung || Cancer of the Lung || Carcinoma of Lung || Carcinoma of the Lung || Lung Cancer A carcinoma originating in the lung. Lung carcinomas usually arise from the epithelium that lines the bronchial tree (bronchogenic carcinomas), and are classified as small cell or non-small cell carcinomas. Non-small cell lung carcinomas are usually adenocarcinomas, squamous cell carcinomas, or large cell carcinomas. Metastatic carcinomas to the lung are also common, and can be difficult to distinguish from primary tumors. Malignant C45543 Lung Carcinosarcoma Pulmonary Carcinosarcoma A morphologic variant of lung sarcomatoid carcinoma composed of a mixture of non-small cell lung carcinoma and a sarcomatous component. Malignant C45512 Lung Colloid Adenocarcinoma Lung Mucinous Cystadenocarcinoma || Lung Mucinous Cystic Tumor of Borderline Malignancy A morphologic variant of lung adenocarcinoma characterized by the presence of mucin pools containing islands of well differentiated adenocarcinoma cells. Malignant C45545 Lung Epithelial-Myoepithelial Carcinoma A lung carcinoma arising within the bronchi or bronchial tubes. It is characterized by the presence of myoepithelial cells, spindle cells, clear cells, and duct-forming epithelial cells. Surgical resection may be curative. Malignant C45509 Lung Fetal Adenocarcinoma Fetal Adenocarcinoma || Pulmonary Adenocarcinoma of Fetal Type || Pulmonary Endodermal Tumor Resembling Fetal Lung || Well-Differentiated Fetal Lung Adenocarcinoma A rare morphologic variant of lung adenocarcinoma characterized by the presence of glandular structures containing glycogen-rich cells forming tubules that resemble fetal lung tubules. Malignant C4452 Lung Giant Cell Carcinoma Giant Cell Carcinoma of Lung || Giant Cell Carcinoma of the Lung A morphologic variant of lung sarcomatoid carcinoma characterized by the presence of mononuclear and multinucleated pleomorphic neoplastic giant cells that lack cohesion. Malignant C4450 Lung Large Cell Carcinoma Anaplastic Lung Carcinoma || Large Cell Carcinoma of Lung || Large Cell Carcinoma of the Lung || Large Cell Lung Carcinoma || Large Cell Undifferentiated Lung Carcinoma An undifferentiated non-small cell lung carcinoma composed of large polygonal cells without evidence of glandular, squamous, or neuroendocrine differentiation. Malignant C6876 Lung Large Cell Carcinoma with Rhabdoid Phenotype Large Cell Carcinoma with Rhabdoid Phenotype || Large Cell Lung Carcinoma with Rhabdoid Phenotype A large cell lung carcinoma characterized by the presence of rhabdoid cells. Malignant C4451 Lung Large Cell Carcinoma, Clear Cell Variant Clear Cell Carcinoma of Lung, Large Cell Type || Clear Cell Carcinoma of the Lung, Large Cell Type || Clear Cell Lung Carcinoma, Large Cell Type || Large Cell Lung Carcinoma, Clear Cell Variant A large cell lung carcinoma characterized by a predominance of clear cells that may or may not contain glycogen. Malignant C5672 Lung Large Cell Neuroendocrine Carcinoma LCNEC of the Lung || Large Cell Lung Carcinoma with Neuroendocrine Differentiation || Large Cell Lung Neuroendocrine Carcinoma || Large Cell Neuroendocrine Carcinoma of Lung || Large Cell Neuroendocrine Carcinoma of the Lung || Pulmonary Large Cell Neuroendocrine Carcinoma A high grade carcinoma that arises from the lung and is characterized by the presence of large neuroendocrine cells. It usually has an aggressive clinical course. Malignant C45519 Lung Lymphoepithelioma-Like Carcinoma Lymphoepithelioma-Like Lung Carcinoma A rare and distinctive type of lung carcinoma characterized by the presence of a syncytial growth pattern, large vesicular nuclei with eosinophilic nucleoli, and dense lymphoplasmacytic infiltration. Malignant C45604 Lung Mucinous Cystadenoma A very rare, well circumscribed, benign cystic neoplasm that arises from the lung. It is characterized by the presence of cysts which are lined by tall mucinous epithelium and filled with mucin. Benign C45544 Lung Mucoepidermoid Carcinoma A lung carcinoma characterized by the presence of malignant non-keratinizing squamoid cells, mucin-producing cells and intermediate type cells. Malignant C5664 Lung Mucous Gland Adenoma Adenoma of Bronchial Mucous Gland || Adenoma of the Bronchial Mucous Gland || Bronchial Cystadenoma || Lung Polyadenoma A benign adenomatous neoplasm that arises from the mucous glands in the bronchus. Benign C2926 Lung Non-Small Cell Carcinoma NSCLC - Non-Small Cell Lung Cancer || Non Small Cell Lung Cancer NOS || Non-Small Cell Cancer of Lung || Non-Small Cell Cancer of the Lung || Non-Small Cell Carcinoma of Lung || Non-Small Cell Carcinoma of the Lung || Non-Small Cell Lung Cancer || Non-Small Cell Lung Carcinoma A group of at least three distinct histological types of lung cancer, including non-small cell squamous cell carcinoma, adenocarcinoma, and large cell carcinoma. Non-small cell lung carcinomas have a poor response to conventional chemotherapy. Malignant C5650 Lung Papillary Adenocarcinoma Papillary Adenocarcinoma of Lung || Papillary Adenocarcinoma of the Lung || Papillary Lung Adenocarcinoma A morphologic variant of lung adenocarcinoma characterized by the presence of papillary structures. Malignant C3494 Lung Papillary Adenoma Adenoma of Bronchus || Adenoma of the Bronchus || Bronchial Adenoma || Papillary Adenoma of Type II Pneumocytes || Peripheral Papillary Tumor of Type II Pneumocytes || Type II Pneumocyte Adenoma A benign lung neoplasm characterized by the presence of a fibrovascular stroma lined by cuboidal to columnar cells. Patients are usually asymptomatic and it is incidentally discovered as a pulmonary nodule during chest X-ray examination. Surgical excision is curative. Benign C45603 Lung Pleomorphic Adenoma A very rare, well circumscribed, benign epithelial neoplasm that arises from the bronchus. It is characterized by the presence of epithelial cells, myoepithelial cells, and fibromyxoid stroma. Benign C45542 Lung Pleomorphic Carcinoma A morphologic variant of sarcomatoid carcinoma characterized by the presence of malignant glandular or squamous cells associated with malignant giant and spindle cells. Malignant C45540 Lung Sarcomatoid Carcinoma A rare, aggressive, poorly differentiated, non-small cell lung carcinoma characterized by the presence of a sarcomatoid component often associated with giant cell differentiation. There is a male to female ratio of 4:1. Clinical symptoms include cough, hemoptysis, chest pain, progressive dyspnea and fever secondary to recurrent pneumonia. Cigarette smoking is a major risk factor. Malignant C4917 Lung Small Cell Carcinoma Lung Small Cell Neuroendocrine Carcinoma || Small Cell Carcinoma of Lung || Small Cell Carcinoma of the Lung || Small Cell Lung Cancer || Small Cell Lung Carcinoma || Small Cell Neuroendocrine Carcinoma of Lung || Small Cell Neuroendocrine Carcinoma of the Lung A highly aggressive subtype of lung carcinoma characterized by the presence of malignant small cells and necrosis. Metastatic disease is usually present at the time of diagnosis. Malignant C45541 Lung Spindle Cell Carcinoma A morphologic variant of sarcomatoid carcinoma characterized by the presence of malignant spindle cells and focal lymphoplasmacytic infiltrates. Adenocarcinoma cells, malignant squamous cells, and giant cells are not present. Malignant C3493 Lung Squamous Cell Carcinoma Epidermoid Cell Carcinoma of Lung || Epidermoid Cell Carcinoma of the Lung || Epidermoid Cell Lung Carcinoma || Squamous Cell Carcinoma of Lung || Squamous Cell Carcinoma of the Lung || Squamous Cell Lung Carcinoma A carcinoma arising from squamous bronchial epithelial cells. It may be keratinizing or non-keratinizing. Keratinizing squamous cell carcinoma is characterized by the presence of keratinization, pearl formation, and/or intercellular bridges. Non-keratinizing squamous cell carcinoma is characterized by the absence of keratinization, pearl formation, and intercellular bridges. Cigarette smoking and arsenic exposure are strongly associated with squamous cell lung carcinoma. Malignant C45503 Lung Squamous Cell Carcinoma, Clear Cell Variant A morphologic variant of squamous cell lung carcinoma characterized by the presence of clear cells. Malignant C45502 Lung Squamous Cell Carcinoma, Papillary Variant A morphologic variant of squamous cell lung carcinoma characterized by the presence of papillary structures. Malignant C45504 Lung Squamous Cell Carcinoma, Small Cell Variant Squamous Cell Lung Carcinoma, Small Cell Variant A poorly differentiated morphologic variant of squamous cell lung carcinoma characterized by the presence of small tumor cells with focal squamous differentiation. Malignant C8965 Lymphangioma A benign lesion composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation. Benign C3205 Lymphangiosarcoma Lymphangioendothelial Sarcoma || Malignant Lymphangioendothelioma A malignant neoplasm arising from the endothelial cells of the lymphatic vessels. Malignant C9360 Lymphoblastic Lymphoma Precursor Cell Lymphoblastic Lymphoma || Precursor Lymphoblastic Lymphoma A lymphoma composed of immature small to medium-sized precursor lymphoid cells (lymphoblasts). It includes the B- and T-cell lymphoblastic lymphoma. Malignant C9283 Lymphocyte-Depleted Classic Hodgkin Lymphoma Hodgkin Lymphoma Lymphocyte Depleted || Hodgkin's Disease Lymphocyte Depletion || Hodgkin's Lymphoma Lymphocyte Depleted || Lymphocyte Depleted Classical Hodgkin Lymphoma || Lymphocyte Depleted Classical Hodgkin's Lymphoma || Lymphocyte Depleted Hodgkin Lymphoma || Lymphocyte Depleted Hodgkin's Disease || Lymphocyte Depleted Hodgkin's Lymphoma || Lymphocyte-Depleted Classical Hodgkin Lymphoma || Lymphocyte-Depleted Hodgkin's Disease || Lymphocyte-Depleted Hodgkin's Lymphoma A diffuse subtype of classic Hodgkin lymphoma which is rich in Hodgkin and Reed-Sternberg cells and/or depleted in non-neoplastic lymphocytes. (WHO, 2008) Malignant C6913 Lymphocyte-Rich Classic Hodgkin Lymphoma Lymphocyte Rich Classical Hodgkin Lymphoma || Lymphocyte Rich Classical Hodgkin's Disease || Lymphocyte Rich Classical Hodgkin's Lymphoma || Lymphocyte Rich Hodgkin Lymphoma || Lymphocyte Rich Hodgkin's Disease || Lymphocyte Rich Hodgkin's Lymphoma || Lymphocyte-Rich Classical Hodgkin Lymphoma || Lymphocyte-Rich Classical Hodgkin's Lymphoma A subtype of classic Hodgkin lymphoma with scattered Hodgkin and Reed-Sternberg cells and a nodular or less often diffuse cellular background consisting of small lymphocytes and with an absence of neutrophils and eosinophils. (WHO, 2008) Malignant C7205 Lymphoepithelioid Variant Peripheral T-Cell Lymphoma Lennert Lymphoma || Lennert Variant Peripheral T-Cell Lymphoma || Lennert's Lymphoma || Lennert's Variant Peripheral T-Cell Lymphoma || Lymphoepithelioid Cell Variant Peripheral T-Cell Lymphoma A variant of peripheral T-cell lymphoma, not otherwise specified. It is characterized by the presence of neoplastic small lymphocytes infiltrating the lymph nodes in a diffuse and less frequently interfollicular pattern. There is an associated proliferation of epithelioid histiocytes forming confluent clusters. Malignant C3208 Lymphoma Lymphoma (Hodgkin and Non-Hodgkin) || Lymphoma (Hodgkin's and Non-Hodgkin's) || Malignant Lymphoma A malignant (clonal) proliferation of B- lymphocytes or T- lymphocytes which involves the lymph nodes, bone marrow and/or extranodal sites. This category includes Non-Hodgkin lymphomas and Hodgkin lymphomas. Malignant C7930 Lymphomatoid Granulomatosis An angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, comprised of Epstein-Barr virus (EBV)-positive B-cells admixed with reactive T-cells. Incidence is higher among adult males; patients with a history of immunodeficiency are at increased risk. The most common site of involvement is the lung; other common sites include brain, kidney, liver, and skin. Morphologically, three grades are recognized: grade I, II, and III. Grade III lymphomatoid granulomatosis should be approached clinically as a subtype of diffuse large B-cell lymphoma. Uncertain Malignant Potential C3721 Lymphomatoid Papulosis A chronic, recurrent cutaneous disorder characterized by the presence of spontaneously regressing papules. The papules are composed of an atypical lymphocytic infiltrate that contains anaplastic CD30-positive T-cells, which are found in type A and diffuse large cell type (type C) lymphomatoid papulosis. In a small number of cases, of type B, the lymphocytic infiltrate is composed of small, cerebriform-like lymphocytes that are often negative for CD30. The majority of cases follow a benign clinical course, but some cases are clonal and may progress to lymphoma. Treatment options include low dose methotrexate and psoralen/UVA (PUVA). Uncertain Malignant Potential C4720 Lymphoplasmacyte-Rich Meningioma A WHO grade I meningioma characterized by the presence of prominent chronic inflammatory infiltrates that predominate over the meningioma cells. Undetermined C3212 Lymphoplasmacytic Lymphoma Lymphoplasmacytoid Lymphoma A clonal neoplasm of small B-lymphocytes, lymphoplasmacytoid cells, and plasma cells involving the bone marrow, lymph nodes, and the spleen. The majority of patients have a serum IgM paraprotein. Malignant C3862 Male Breast Carcinoma Carcinoma of Male Breast || Carcinoma of the Male Breast || Male Breast Cancer A rare malignant tumor of the breast, affecting mostly older men. It accounts for less than 1% of all malignancies in men. The most common histologic type is invasive ductal carcinoma. Most patients are treated with mastectomy. Malignant C4220 Malignant Adrenal Gland Pheochromocytoma Malignant Adrenal Gland Chromaffin Neoplasm || Malignant Adrenal Gland Chromaffin Paraganglioma || Malignant Adrenal Gland Chromaffin Tumor || Malignant Adrenal Gland Chromaffinoma || Malignant Adrenal Gland Paraganglioma || Malignant Adrenal Medullary Paraganglioma || Malignant Adrenal Medullary Pheochromocytoma || Malignant Adrenal Pheochromocytoma || Malignant Pheochromocytoma || Pheochromoblastoma A pheochromocytoma that metastasizes to other anatomic sites. Common sites of metastasis include lymph nodes, bones, liver, and lung. Morphologic features associated with malignant pheochromocytomas include: atypical mitotic figures, capsular and vascular invasion, tumor cell necrosis, and high mitotic activity. Malignant C4504 Malignant Breast Phyllodes Tumor Malignant Breast Phyllodes Neoplasm || Malignant Mammary Phyllodes Neoplasm || Malignant Mammary Phyllodes Tumor || Malignant Phyllodes Breast Neoplasm || Malignant Phyllodes Neoplasm of Breast || Malignant Phyllodes Neoplasm of the Breast || Malignant Phyllodes Tumor of Breast || Malignant Phyllodes Tumor of the Breast A phyllodes tumor of the breast characterized by infiltrative margins and a sarcomatous stromal component. The sarcomatous stroma usually displays features of fibrosarcoma. Liposarcomatous, osteosarcomatous, or rhabdomyosarcomatous elements may also be present. Malignant C8881 Malignant Extragonadal Germ Cell Tumor Malignant Neoplasm of Extragonadal Germ Cell || Malignant Neoplasm of the Extragonadal Germ Cell || Malignant Tumor of Extragonadal Germ Cell || Malignant Tumor of the Extragonadal Germ Cell A malignant germ cell tumor that develops as a primary tumor in an anatomic site other than the testis or ovary. Malignant C65188 Malignant Gastrinoma A gastrin-producing neuroendocrine tumor. It is characterized by inappropriate secretion of gastrin and associated with Zollinger Ellison syndrome. It displays vascular invasion and metastasizes to other anatomic sites. Malignant C53999 Malignant Gastrointestinal Stromal Tumor A gastrointestinal stromal tumor that is characterized by large size (diameter greater than 10 cm for gastric localization and greater than 5 cm for intestinal localization) or more than 5 mitotic figures per 50 high power fields. Malignant C4925 Malignant Germ Cell Tumor Malignant Germ Cell Neoplasm || Malignant Neoplasm of Germ Cell || Malignant Neoplasm of the Germ Cell || Malignant Tumor of Germ Cell || Malignant Tumor of the Germ Cell A gonadal or extragonadal malignant neoplasm that arises from germ cells. Representative examples include embryonal carcinoma, yolk sac tumor, and seminoma. Malignant C4822 Malignant Glioma High Grade Glioma || High-Grade Glioma || Malignant Glial Neoplasm || Malignant Glial Tumor || Malignant Neuroglial Neoplasm || Malignant Neuroglial Tumor A grade III or grade IV glioma arising from the central nervous system. This category includes glioblastoma, anaplastic astrocytoma, anaplastic ependymoma, anaplastic oligodendroglioma, and anaplastic oligoastrocytoma. Malignant C4221 Malignant Glomus Tumor Glomangiosarcoma || Malignant Glomus Neoplasm A very rare morphologic variant of glomus tumor with a size greater than 2 cm. The tumor arises in subfascial or visceral tissues. It is characterized by the presence of atypical mitotic figures, or marked nuclear atypia, or the combination of both. It has an aggressive clinical course. Malignant C4336 Malignant Granular Cell Tumor Malignant Granular Cell Myoblastoma || Malignant Granular Cell Neoplasm An uncommon granular cell tumor which may metastasize to other anatomic sites. Morphologic characteristics include the presence of spindling neoplastic cells, necrosis, extensive pleomorphism, prominent nucleoli, and increased mitiotic activity. Malignant C4748 Malignant Melanotic Peripheral Nerve Sheath Tumor Malignant Melanocytic Neoplasm of Peripheral Nerve Sheath || Malignant Melanocytic Neoplasm of the Peripheral Nerve Sheath || Malignant Melanocytic Peripheral Nerve Sheath Tumor || Malignant Melanotic Nerve Sheath Tumor || Melanocytic MPNST A rare variant of malignant peripheral nerve sheath tumor. It is characterized by the presence of malignant cells that contain melanin. Malignant C6910 Malignant Melanotic Psammomatous Peripheral Nerve Sheath Tumor Melanocytic Psammomatous MPNST || Melanocytic Psammomatous Malignant Peripheral Nerve Sheath Tumor || Melanotic Psammomatous Malignant Peripheral Nerve Sheath Tumor A malignant peripheral nerve sheath tumor characterized by the presence of malignant cells that contain melanin and formation of psammoma bodies. Malignant C4456 Malignant Mesothelioma Malignant Mesothelial Neoplasm || Malignant Mesothelial Tumor || Malignant Neoplasm of Mesothelium || Malignant Neoplasm of the Mesothelium || Malignant Tumor of Mesothelium || Malignant Tumor of the Mesothelium A malignant neoplasm that arises from mesothelial cells. It is associated with exposure to asbestos. Malignant C8975 Malignant Mixed Mesodermal (Mullerian) Tumor Malignant Mixed Mesodermal (Müllerian) Tumor || Malignant Mixed Mesodermal Tumor || Malignant Mixed Mullerian Tumor || Malignant Mixed Müllerian Tumor An aggressive malignant tumor of the female reproductive system, affecting predominantly elderly menopausal women. The endometrium and ovary are the most common sites of tumor origin. Morphologically, it is a high grade tumor, composed of carcinomatous and sarcomatous elements. Malignant C121933 Malignant Osteoclastic Giant Cell-Rich Tumor of Bone A giant cell tumor that arises from the bone and is characterized by the presence of a malignant cellular component. Malignant C4514 Malignant Ovarian Germ Cell Tumor Malignant Germ Cell Neoplasm of Ovary || Malignant Germ Cell Neoplasm of the Ovary || Malignant Germ Cell Tumor of Ovary || Malignant Germ Cell Tumor of the Ovary || Malignant Ovarian Germ Cell Neoplasm || Ovarian Germ Cell Cancer An invasive malignant neoplasm that arises from the ovary and originates from primordial germ cells. Representative examples include embryonal carcinoma, yolk sac tumor, dysgerminoma, and non-gestational choriocarcinoma. Malignant C3798 Malignant Peripheral Nerve Sheath Tumor Malignant Neoplasm of Peripheral Nerve Sheath || Malignant Neoplasm of the Peripheral Nerve Sheath || Malignant Neurilemmoma || Malignant Neurilemoma || Malignant Peripheral Nerve Sheath Neoplasm || Malignant Peripheral Nerve Sheath Tumour || Malignant Schwannoma || Malignant Tumor of Peripheral Nerve Sheath || Malignant Tumor of the Peripheral Nerve Sheath || Neurofibrosarcoma || Neurogenic Sarcoma An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region. It may be associated with neurofibromatosis 1 (NF1). Malignant C92647 Malignant Peripheral Nerve Sheath Tumor with Mesenchymal Differentiation A malignant peripheral nerve sheath tumor characterized by the presence of mesenchymal differentiation. Representative example is the malignant Triton tumor which contains a rhabdomyosarcomatous component. Malignant C4275 Malignant Phyllodes Tumor Malignant Cystosarcoma Phyllodes || Malignant Phyllodes Neoplasm A phyllodes tumor with sarcomatous stroma. The sarcomatous component is usually of the fibrosarcomatous type. Liposarcomatous, chondrosarcomatous, osteosarcomatous, or rhabdomyosarcomatous differentiation may also occur in the stroma. It may recur and metastasize following surgical resection. The lung and skeleton are the anatomic sites most frequently involved by metastases. Malignant C5531 Malignant Prostate Phyllodes Tumor Malignant Phyllodes Neoplasm of Prostate || Malignant Phyllodes Neoplasm of the Prostate || Malignant Phyllodes Tumor of Prostate || Malignant Phyllodes Tumor of the Prostate || Malignant Prostate Phyllodes Neoplasm An unusual malignant tumor that arises from the prostate gland. It is characterized by the presence of glandular elements and a cellular stroma that exhibits mitotic activity and nuclear atypia. Malignant C6894 Malignant Solitary Fibrous Tumor A malignant neoplasm of probable fibroblastic derivation. It is characterized by the presence of atypical round to spindle-shaped cells, increased cellularity, necrotic change and high mitotic activity. Malignant C65190 Malignant Somatostatinoma Delta Cell Carcinoma A malignant neuroendocrine tumor arising from delta cells which produce somatostatin. It displays vascular invasion and metastasizes to other anatomic sites. Malignant C6535 Malignant Tenosynovial Giant Cell Tumor Malignant Giant Cell Neoplasm of Tendon Sheath || Malignant Giant Cell Neoplasm of the Tendon Sheath || Malignant Giant Cell Tumor of Tendon Sheath || Malignant Giant Cell Tumor of the Tendon Sheath || Malignant Tendon Sheath Giant Cell Neoplasm || Malignant Tendon Sheath Giant Cell Tumor An uncommon malignant tumor arising from the tendon sheath. Morphologically, it is characterized by the presence of a cellular infiltrate reminiscent of a giant cell tumor with prominent malignant characteristics. Recurrent giant cell tumors with a sarcomatous dedifferentiation are included in this category as well. Malignant C9063 Malignant Testicular Germ Cell Tumor Malignant Germ Cell Neoplasm of Testis || Malignant Germ Cell Neoplasm of the Testis || Malignant Germ Cell Tumor of Testis || Malignant Germ Cell Tumor of the Testis || Malignant Testicular Germ Cell Neoplasm || Testicular Cancer || Testicular Germ Cell Cancer A malignant tumor predominantly affecting young men and often associated with cryptorchidism. Seminoma is the most frequently seen malignant testicular germ cell tumor, followed by embryonal carcinoma and yolk sac tumor. Malignant C7612 Malignant Thymoma A thymoma that has an aggressive clinical course (capsular invasion, infiltration of the surrounding tissues) and can metastasize. Although any morphologic subtype of thymoma may eventually have a malignant clinical course, this term is most often associated with thymoma types B3 and C. Malignant C4335 Malignant Triton Tumor MPNST with Rhabdomyosarcoma || Malignant Neoplasm of Peripheral Nerve Sheath with Rhabdomyosarcoma || Malignant Neoplasm of the Peripheral Nerve Sheath with Rhabdomyosarcoma || Malignant Peripheral Nerve Sheath Neoplasm with Rhabdomyosarcoma || Malignant Peripheral Nerve Sheath Tumor with Rhabdomyosarcoma || Malignant Tumor of Peripheral Nerve Sheath with Rhabdomyosarcoma || Malignant Tumor of the Peripheral Nerve Sheath with Rhabdomyosarcoma A malignant peripheral nerve sheath tumor which shows rhabdomyosarcomatous differentiation. More than half of the patients have neurofibromatosis type 1. The prognosis is usually poor. Malignant C7999 Malignant Type A Thymoma A type A thymoma which is characterized by an aggressive clinical course (capsular invasion, infiltration of the surrounding tissues) and can metastasize. Malignant C6886 Malignant Type AB Thymoma A type AB thymoma which is characterized by an aggressive clinical course (capsular invasion, infiltration of the surrounding tissues) and can metastasize. Malignant C7996 Malignant Type B1 Thymoma A type B1 thymoma which is characterized by an aggressive clinical course (capsular invasion, infiltration of the surrounding tissues) and can metastasize. Malignant C6889 Malignant Type B2 Thymoma A type B2 thymoma which is characterized by an aggressive clinical course (capsular invasion, infiltration of the surrounding tissues) and can metastasize. Malignant C65189 Malignant Vipoma An aggressive neuroendocrine tumor located in the pancreas or small intestine. It is composed of cells containing vasoactive intestinal peptide. It is associated with watery diarrhea, hypokalemia, and hypochlorhydria or achlorhydria. It displays vascular invasion and metastasizes to other anatomic sites. Malignant C45928 Mammosomatotroph Adenoma Mammosomatotroph Pituitary Gland Adenoma A pituitary gland adenoma composed of acidophilic cells that produce both growth hormone and prolactin. Immunohistochemical studies reveal the presence of these two hormones localized in the same cell. Benign C4337 Mantle Cell Lymphoma Classical Mantle Cell Lymphoma An aggressive, usually diffuse non-Hodgkin lymphoma composed of small to medium sized B-lymphocytes (centrocytes). Most patients present with advanced stage disease with lymphadenopathy, hepatosplenomegaly, and bone marrow involvement. The gastrointestinal tract is the most commonly affected extranodal site by this type of non-Hodgkin lymphoma. The vast majority of cases express the t(11;14)(q13;q32) resulting in the rearrangement of the BCL-1 gene and the overexpression of cyclin D1 mRNA. Malignant C4341 Marginal Zone Lymphoma Marginal Zone B-Cell Lymphoma A usually indolent mature B-cell lymphoma, arising from the marginal zone of lymphoid tissues. It is characterized by the presence of small to medium sized atypical lymphocytes. It comprises three entities, according to the anatomic sites involved: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, which affects extranodal sites (most often stomach, lung, skin, and ocular adnexa); nodal marginal zone B-cell lymphoma, which affects lymph nodes without evidence of extranodal disease; and splenic marginal zone B-cell lymphoma, which affects the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood. Malignant C3169 Mast Cell Leukemia A variant of systemic mastocytosis with involvement of the bone marrow (20% or more mast cells) and the peripheral blood (mast cells account for 10% or more of peripheral blood white cells). (WHO, 2001) Malignant C9348 Mast Cell Sarcoma A rare malignant neoplasm characterized by localized but destructive growth of a tumor consisting of highly atypical, immature mast cells. (WHO, 2001) Malignant C3468 Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma Mature T-Cell Non-Hodgkin's Lymphoma || Mature T-Cell and NK-Cell Lymphoma || Mature T-Cell and NK-Cell Non-Hodgkin's Lymphoma || Mature T-and NK-Cell Lymphoma || Peripheral T-Cell Lymphoma This type of lymphoma is not frequently seen in the western hemisphere. Clinically, with the exception of anaplastic large cell lymphoma, mature T- and NK-cell lymphomas are among the most aggressive of all hematopoietic neoplasms. Representative disease entities include mycosis fungoides, angioimmunoblastic T-cell lymphoma, hepatosplenic T-cell lymphoma, and anaplastic large cell lymphoma. Malignant C9015 Mature Teratoma Grade 0 Teratoma A teratoma which may be cystic; it is composed entirely of well differentiated, adult-type mature tissues, without evidence of fetal-type immature tissues (grade 0 teratoma). Benign C3222 Medulloblastoma A malignant, invasive embryonal neoplasm arising from the cerebellum or posterior fossa. It occurs predominantly in children and has the tendency to metastasize via the cerebrospinal fluid pathways. Signs and symptoms include truncal ataxia, disturbed gait, lethargy, headache, and vomiting. There are four histologic variants: classic medulloblastoma, large cell/anaplastic medulloblastoma, desmoplastic/nodular medulloblastoma, and medulloblastoma with extensive nodularity. Malignant C5407 Medulloblastoma with Extensive Nodularity Cerebellar Neuroblastoma || Medulloblastoma with Extensive Nodularity and Advanced Neuronal Differentiation || Nodular Medulloblastoma A medulloblastoma characterized by nodularity and neuronal differentiation. Malignant C129444 Medulloblastoma, Non-WNT/Non-SHH Medulloblastoma not associated with activation of the WNT pathway or sonic hedgehog (SHH) pathway. TP53 mutations are absent. This molecular subtype includes medulloblastomas numerically designated as "group 3" and "group 4". Malignant C129441 Medulloblastoma, SHH-Activated A molecular subtype of medulloblastoma associated with activation of the sonic hedgehog (SHH) pathway. TP53 mutations may be present or absent. Patients in this group are young children and adults. Overall survival is variable and depends on the presence or absence of metastatic disease, histology, and the age at diagnosis. Malignant C129442 Medulloblastoma, SHH-Activated, TP53-Mutant Medulloblastoma associated with activation of the sonic hedgehog (SHH) pathway and the presence of TP53 mutations. Malignant C129443 Medulloblastoma, SHH-Activated, TP53-Wildtype Medulloblastoma associated with activation of the sonic hedgehog (SHH) pathway and the absence of TP53 mutations. Malignant C129440 Medulloblastoma, WNT-Activated A molecular subtype of medulloblastoma associated with activation of the WNT pathway. TP53 mutations may be present or absent. WNT pathway activation in medulloblastomas is associated with good outcome. Malignant C3224 Melanoma Malignant Melanoma A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Most often, melanomas arise in the skin (cutaneous melanomas) and include the following histologic subtypes: superficial spreading melanoma, nodular melanoma, acral lentiginous melanoma, and lentigo maligna melanoma. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas may also arise in other anatomic sites including the gastrointestinal system, eye, urinary tract, and reproductive system. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain. Malignant C4660 Meningeal Solitary Fibrous Tumor/Hemangiopericytoma A solitary fibrous tumor/hemangiopericytoma that arises from the meninges. Undetermined C92652 Meningeal Solitary Fibrous Tumor/Hemangiopericytoma, Grade 3 A solitary fibrous tumor/hemangiopericytoma that arises from the meninges. It most often corresponds to the tumor previously diagnosed as anaplastic hemangiopericytoma. Malignant C3230 Meningioma A generally slow growing tumor attached to the dura mater. It is composed of neoplastic meningothelial (arachnoidal) cells. It typically occurs in adults, often women and it has a wide range of histopathological appearances. Of the various subtypes, meningothelial, fibrous and transitional meningiomas are the most common. Most meningiomas are WHO grade I tumors, and some are WHO grade II or III tumors. Most subtypes share a common clinical behavior, although some subtypes are more likely to recur and follow a more aggressive clinical course. (Adapted from WHO) Undetermined C9231 Merkel Cell Carcinoma Cutaneous Apudoma || Cutaneous Neuroendocrine Carcinoma || Neuroendocrine Carcinoma of Skin || Neuroendocrine Carcinoma of the Skin || Neuroendocrine Skin Carcinoma || Trabecular Skin Carcinoma A rare malignant cutaneous tumor seen in elderly patients. Its usual location is on the head, neck and extremities. The tumor is composed of small round cells with scanty cytoplasm arranged in a trabecular pattern, or in ill-defined nodules or in a diffuse pattern. The tumor cells contain cytoplasmic membrane-bound dense core granules resembling neurosecretory granules. There is strong evidence implicating Merkel cell polyomavirus in a majority of cases of Merkel cell carcinoma. Malignant C3737 Mesenchymal Chondrosarcoma A morphologic variant of chondrosarcoma arising from bone and soft tissue. It is characterized by the presence of malignant small round cells, biphasic growth pattern, and well differentiated hyaline cartilage. Clinical presentation includes pain and swelling. The clinical course is aggressive, with local recurrences and distant metastases. Malignant C111695 Mesenchymal Glioblastoma A molecular subtype of glioblastoma characterized by the presence of NF1 mutations. Malignant C3234 Mesothelioma A usually malignant and aggressive neoplasm of the mesothelium which is often associated with exposure to asbestos. Undetermined C38158 Metachronous Kidney Wilms Tumor Metachronous Kidney Nephroblastoma || Metachronous Wilms Tumor || Metachronous Wilms Tumor of the Kidney Wilms tumor arising in the remaining kidney following treatment of the original Wilms tumor. Malignant C38157 Metachronous Osteosarcoma Metachronous Osteosarcoma of the Bone A bone osteosarcoma that has metastasized to skeletal or extraskeletal sites. Malignant C6907 Metaplastic Meningioma A WHO grade I meningioma characterized by the presence of a prominent mesenchymal component. The mesenchymal component may be osseous, cartilaginous, myxoid, lipomatous, or a mixture of mesenchymal elements. Undetermined C4721 Microcystic Meningioma A WHO grade I meningioma characterized by the presence of intercellular microcystic spaces that contain mucinous fluid. Undetermined C27757 Microgranular Acute Promyelocytic Leukemia Hypogranular Acute Promyelocytic Leukemia Acute promyelocytic leukemia in which the promyelocytes in the peripheral blood have paucity or absence of cytoplasmic granules and characteristic bilobed nuclei. Malignant C6089 Middle Ear Carcinoma Carcinoma of Middle Ear || Carcinoma of the Middle Ear A carcinoma that arises from the middle ear. This category includes adenocarcinoma and squamous cell carcinoma. Malignant C2923 Minimally Invasive Lung Adenocarcinoma A solitary adenocarcinoma arising from the lung and measuring 3 cm or less in size. It is characterized by a predominantly lepidic pattern and 5 mm or less invasion in greatest dimension. It is usually a non-mucinous adenocarcinoma, but rarely may be mucinous. Malignant C5948 Minor Salivary Gland Adenocarcinoma Adenocarcinoma of Minor Salivary Gland || Adenocarcinoma of the Minor Salivary Gland An adenocarcinoma that arises from the minor salivary glands. Malignant C5936 Minor Salivary Gland Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Minor Salivary Gland || Adenoid Cystic Carcinoma of the Minor Salivary Gland An aggressive carcinoma that arises from the minor salivary glands. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Malignant C5957 Minor Salivary Gland Carcinoma Carcinoma of Minor Salivary Gland || Carcinoma of the Minor Salivary Gland A carcinoma that arises from the minor salivary glands. Representative examples include adenoid cystic carcinoma, acinic cell carcinoma, polymorphous low grade adenocarcinoma, and mucinous adenocarcinoma. Malignant C5976 Minor Salivary Gland Carcinoma ex Pleomorphic Adenoma Carcinoma ex Pleomorphic Adenoma of Minor Salivary Gland || Carcinoma ex Pleomorphic Adenoma of the Minor Salivary Gland A carcinoma that arises from a pleomorphic adenoma in the minor salivary glands. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive. Malignant C5953 Minor Salivary Gland Mucoepidermoid Carcinoma Mucoepidermoid Carcinoma of Minor Salivary Gland || Mucoepidermoid Carcinoma of the Minor Salivary Gland A carcinoma that arises from the minor salivary glands. It usually presents as a firm and painless mass. It is characterized by the presence of epidermoid cells, mucus producing cells, and cells of intermediate type. The majority of cases have a favorable outcome. Malignant C5956 Minor Salivary Gland Small Cell Neuroendocrine Carcinoma Minor Salivary Gland Neuroendocrine Carcinoma || Neuroendocrine Carcinoma of Minor Salivary Gland || Neuroendocrine Carcinoma of the Minor Salivary Gland An infrequent small cell carcinoma that arises from a minor salivary gland. It is characterized by the presence of neuroendocrine differentiation and a high number of mitotic figures. Malignant C5959 Minor Salivary Gland Squamous Cell Carcinoma Epidermoid Carcinoma of Minor Salivary Gland || Epidermoid Carcinoma of the Minor Salivary Gland || Minor Salivary Gland Epidermoid Carcinoma || Squamous Cell Carcinoma of Minor Salivary Gland || Squamous Cell Carcinoma of the Minor Salivary Gland A squamous cell carcinoma that affects the minor salivary glands. Malignant C5954 Minor Salivary Gland Undifferentiated Carcinoma Anaplastic Carcinoma of Minor Salivary Gland || Anaplastic Carcinoma of the Minor Salivary Gland || Anaplastic Minor Salivary Gland Carcinoma || Undifferentiated Carcinoma of Minor Salivary Gland || Undifferentiated Carcinoma of the Minor Salivary Gland A carcinoma that affects the minor salivary glands and is characterized by the presence of undifferentiated, anaplastic malignant epithelial cells. Malignant C8271 Mixed Astrocytoma-Ependymoma A tumor of mixed cell type with astrocytic components as well as ependymoma components. Undetermined C8272 Mixed Astrocytoma-Ependymoma-Oligodendroglioma A mixed glioma characterized by the presence of astrocytic, ependymal, and oligodendroglial neoplastic components. Undetermined C9149 Mixed Cell Type Kidney Wilms Tumor Mixed Cell Type Kidney Adenosarcoma || Mixed Cell Type Kidney Nephroblastoma || Mixed Cell Type Renal Adenosarcoma || Mixed Cell Type Renal Wilm's Tumor || Mixed Cell Type Renal Wilms Tumor || Mixed Cell Type Renal Wilms' Tumor Wilms tumor of the kidney characterized by the presence of blastema, epithelial, and mesenchymal components (triphasic pattern) or a combination of two of them (biphasic pattern). Malignant C3517 Mixed Cellularity Classic Hodgkin Lymphoma Hodgkin's Disease Mixed Cellularity || Hodgkin's Lymphoma Mixed Cellularity || Mixed Cellularity Classical Hodgkin Lymphoma || Mixed Cellularity Hodgkin Lymphoma || Mixed Cellularity Hodgkin's Disease || Mixed Cellularity Hodgkin's Lymphoma A subtype of classic Hodgkin lymphoma with scattered Reed-Sternberg and Hodgkin cells in a diffuse or vaguely nodular mixed inflammatory background without nodular sclerosing fibrosis. (WHO, 2008) Malignant C97058 Mixed Congenital Mesoblastic Nephroma A congenital mesoblastic nephroma characterized by the presence of classic and cellular areas. Malignant C66756 Mixed Epithelioid and Spindle Cell Melanoma A melanoma characterized by the presence of malignant large epithelioid melanocytes and malignant spindle-shaped melanocytes. Malignant C4290 Mixed Germ Cell Tumor Combined Germ Cell Neoplasm || Combined Germ Cell Tumor || Mixed Germ Cell Neoplasm A malignant germ cell tumor characterized by the presence of at least two different germ cell tumor components. The different germ cell tumor components include choriocarcinoma, embryonal carcinoma, yolk sac tumor, teratoma, and seminoma. It occurs in the ovary, testis, and extragonadal sites including central nervous system and mediastinum. Malignant C5241 Mixed Germ Cell-Sex Cord-Stromal Tumor Mixed Germ Cell-Sex Cord Neoplasm || Mixed Germ Cell-Sex Cord Tumor || Mixed Germ Cell-Sex Cord-Stromal Neoplasm A biphasic neoplasm that arises from the ovary or the testis. It is characterized by the presence of neoplastic germ cells and neoplastic sex cord-stromal cells. It includes the gonadoblastoma and mixed germ cell-sex cord stromal tumor, unclassifiable. Undetermined C3903 Mixed Glioma Mixed Glial Neoplasm || Mixed Glial Tumor || Mixed Neuroglial Neoplasm || Mixed Neuroglial Tumor A tumor composed of two or more glial cell types (astrocytes, ependymal cells, and oligodendrocytes). Undetermined C4253 Mixed Liposarcoma A malignant neoplasm characterized by the presence of a combination of liposarcomatous morphologic subtypes: myxoid/round cell and well differentiated/dedifferentiated liposarcoma or myxoid/round cell and pleomorphic liposarcoma. Malignant C82179 Mixed Phenotype Acute Leukemia An acute leukemia of ambiguous lineage. It is characterized by the presence of either separate populations of blasts of more than one lineage, or one population of blasts co-expressing markers of more than one lineage. Malignant C82192 Mixed Phenotype Acute Leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1 Mixed Phenotype Acute Leukemia with t(9;22)(q34;q11.2); BCR-ABL1 A rare mixed phenotype acute leukemia in which the blasts also carry the translocation t(9;22)(q34.1;q11.2) by karyotypic analysis or the BCR-ABL1 translocation by FISH or PCR. The prognosis is usually unfavorable. Malignant C82203 Mixed Phenotype Acute Leukemia with t(v;11q23.3); MLL Rearranged Mixed Phenotype Acute Leukemia with t(v;11q23); MLL Rearranged || Mixed Phenotype Acute Leukemia with t(v;11q23.3); KMT2A Rearranged A rare mixed phenotype acute leukemia in which the blasts carry a translocation between the MLL (KMT2A) gene at 11q23.3 and another gene partner. The prognosis is usually unfavorable. Malignant C82212 Mixed Phenotype Acute Leukemia, B/Myeloid, Not Otherwise Specified Mixed Phenotype Acute Leukemia, B/Myeloid, NOS A rare mixed phenotype acute leukemia in which the blasts express B-lymphoid and myeloid lineage markers but are negative for MLL translocation and t(9;22)(q34;q11.2) translocation. The prognosis is usually unfavorable. Malignant C82213 Mixed Phenotype Acute Leukemia, T/Myeloid, Not Otherwise Specified Mixed Phenotype Acute Leukemia, T/Myeloid, NOS A rare mixed phenotype acute leukemia in which the blasts express T-lymphoid and myeloid lineage markers but are negative for MLL translocation and t(9;22)(q34;q11.2) translocation. The prognosis is usually unfavorable. Malignant C45927 Mixed Somatotroph and Lactotroph Adenoma Mixed GH-PRL Cell Adenoma || Mixed Somatotroph-Lactotroph Adenoma || Mixed Somatotroph-Lactotroph Pituitary Gland Adenoma An infrequent pituitary gland adenoma composed of an admixture of acidophilic and chromophobic cells that produce growth hormone and prolactin respectively. Unlike mammosomatotroph adenomas, these two hormones are not localized in the same cell by immunohistochemistry. Benign C27906 Moderately Differentiated Prostate Adenocarcinoma An invasive prostate adenocarcinoma characterized by the presence of malignant cells exhibiting moderate differentiation. Malignant C35816 Monoblastic Sarcoma A less common form of myeloid sarcoma composed of monoblasts. Monoblastic sarcoma may precede or occur simultaneously with acute monoblastic leukemia. (WHO 2001) Malignant C96058 Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma Monomorphic CD56+ Intestinal T-Cell Lymphoma || Type II EATL A mature T-cell and NK-cell non-Hodgkin lymphoma of intraepithelial T-lymphocytes. It usually arises from the small intestine. A minority of cases arise from the large intestine or the stomach. It is characterized by the presence of a monomorphic cellular infiltrate of small to medium-sized T-lymphocytes that are cytotoxic and express CD56. It is not associated with celiac disease. Malignant C7233 Monomorphic Post-Transplant Lymphoproliferative Disorder Monomorphic PTLD A clonal lymphoproliferative disorder arising as a result of post-transplant immunosuppression therapy. It is characterized by destructive infiltration of lymph nodes and extranodal sites by a monotonous population of transformed lymphocytes or plasma cells. It includes two variants: monomorphic B-cell and monomorphic T/NK cell post-transplant lymphoproliferative disorders. Malignant C3892 Mu Heavy Chain Disease A clonal disorder characterized by the secretion of a mu heavy chain that lacks a variable region. Most patients present with slowly progressive chronic lymphocytic leukemia (CLL). In contrast to most cases of CLL, Mu heavy chain disease is associated with hepatosplenomegaly and absence of lymphadenopathy. Malignant C26712 Mucinous Adenocarcinoma Colloid Adenocarcinoma || Colloid Carcinoma || Gelatinous Adenocarcinoma || Gelatinous Carcinoma || Mucinous Carcinoma || Mucoid Adenocarcinoma || Mucoid Carcinoma || Mucous Adenocarcinoma || Mucous Carcinoma An invasive adenocarcinoma composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation. It may arise from the large and small intestine, appendix, stomach, lung, ovary, breast, corpus uteri, cervix, vagina, and salivary gland. Malignant C39807 Mucinous Tubular and Spindle Cell Carcinoma of the Kidney A low grade carcinoma of the kidney characterized by the presence of tubules which are separated by mucinous stroma. Often the tubular structures have a spindle cell appearance. Patients are usually asymptomatic and occasionally they may present with hematuria or flank pain. Malignant C27379 Mucin-Producing Adenocarcinoma An invasive adenocarcinoma composed of malignant glandular cells which produce mucin. Malignant C3765 Multicystic Mesothelioma Cystic Mesothelioma || Intermediate Mesothelioma An intermediate grade neoplasm arising from mesothelial cells. It occurs in the peritoneum, extraperitoneal space, omentum, or pelvic or abdominal viscera. It is characterized by the presence of multiple cysts lined by flattened or cuboidal mesothelial cells. There is no evidence of significant cytologic atypia or increased mitotic activity. It usually occurs in young to middle-aged women. Patients present with abdominal or pelvic mass and pain. Approximately half of the cases recur. The recurrences may appear several years after the initial diagnosis. Rare cases of transformation to malignant mesothelioma have also been described. Uncertain Malignant Potential C3246 Mycosis Fungoides A peripheral (mature) T-cell lymphoma presenting in the skin with patches/plaques. It is characterized by epidermal and dermal infiltration of small to medium-sized T-cells with cerebriform nuclei. Patients with limited disease generally have an excellent prognosis. In the more advanced stages, the prognosis is poor. (WHO, 2001) Malignant C9265 Mycosis Fungoides and Sezary Syndrome Mycosis Fungoides and Sézary Syndrome Mature T and NK neoplasms predominantly affecting the skin and the peripheral blood. Peripheral blood involvement by abnormal T-cells (cerebriform cells) is a late manifestation of mycosis fungoides, whereas it is the presenting finding in Sezary syndrome. Malignant C3247 Myelodysplastic Syndrome Dysmyelopoietic Syndrome || Hematopoeitic - Myelodysplastic Syndrome (MDS) || Myelodysplasia || Myelodysplastic Neoplasm || Myelodysplastic Syndrome/Neoplasm || Oligoblastic Leukemia || Smoldering Leukemia A clonal hematopoietic disorder characterized by dysplasia and ineffective hematopoiesis in one or more of the hematopoietic cell lines. The dysplasia may be accompanied by an increase in myeloblasts, but the number is less than 20%, which, according to the WHO guidelines, is the requisite threshold for the diagnosis of acute myeloid leukemia. It may occur de novo or as a result of exposure to alkylating agents and/or radiotherapy. (WHO, 2001) Malignant C7506 Myelodysplastic Syndrome with Excess Blasts Refractory Anemia with Excess Blasts || Refractory Anemia with an Excess of Blasts A myelodysplastic syndrome characterized by the presence of 5-19% myeloblasts in the bone marrow or 2-19% blasts in the peripheral blood. It includes two categories: myelodysplastic syndrome with excess blasts-1 and myelodysplastic syndrome with excess blasts-2. Malignant C82595 Myelodysplastic Syndrome with Excess Blasts and Fibrosis RAEB with Fibrosis Myelodysplastic syndrome with excess blasts associated with significant reticulin fibrosis of the bone marrow. Malignant C7167 Myelodysplastic Syndrome with Excess Blasts-1 A myelodysplastic syndrome defined by 5-9% blasts in the bone marrow, and <5% blasts in the blood. Approximately 25% of cases progress to an acute leukemia. (WHO) Malignant C7168 Myelodysplastic Syndrome with Excess Blasts-2 A myelodysplastic syndrome defined by 10-19% blasts in the bone marrow or 5-19% blasts in the blood. Approximately 33% of cases progress to acute leukemia. The prognosis is usually poor. Malignant C6867 Myelodysplastic Syndrome with Isolated del(5q) 5q- Syndrome || Myelodysplastic Syndrome Associated with Isolated del (5q) Chromosome Abnormality || Myelodysplastic Syndrome with 5q Deletion A myelodysplastic syndrome characterized by a deletion between bands q31 and 33 on chromosome 5. The number of blasts in the bone marrow and blood is <5%. The bone marrow is usually hypercellular or normocellular with increased number of often hypolobated megakaryocytes. The peripheral blood shows macrocytic anemia. This syndrome occurs predominantly but not exclusively in middle age to older women. The prognosis is good and transformation to acute leukemia is rare. (WHO, 2001) Malignant C8574 Myelodysplastic Syndrome with Multilineage Dysplasia Refractory Cytopenia with Multilineage Dysplasia A myelodysplastic syndrome characterized by bi-cytopenia or pancytopenia and dysplastic changes in 10% or more of the cells in two or more of the myeloid cell lines. (WHO, 2001) Malignant C4036 Myelodysplastic Syndrome with Ring Sideroblasts Pure Sideroblastic Anemia || Refractory Anemia with Ring Sideroblasts A myelodysplastic syndrome characterized by an anemia in which 15% or more of the erythroid precursors are ring sideroblasts. The ring sideroblast is an erythroid precursor in which one third or more of the nucleus is encircled by granules which are positive for iron stain. (WHO, 2001) Malignant C27726 Myelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia Refractory Cytopenia with Multilineage Dysplasia and Ring Sideroblasts A myelodysplastic syndrome characterized by bi-cytopenia or pancytopenia, dysplastic changes in 10% or more of the cells in two or more of the myeloid cell lines, and 15% or more ring sideroblasts in the bone marrow. (WHO, 2001) Malignant C130037 Myelodysplastic Syndrome with Ring Sideroblasts and Single Lineage Dysplasia A myelodysplastic syndrome with ring sideroblasts and dysplastic changes involving only one myeloid cell lineage in the bone marrow. Malignant C82591 Myelodysplastic Syndrome with Single Lineage Dysplasia Refractory Cytopenia with Unilineage Dysplasia A myelodysplastic syndrome characterized by dysplastic changes involving only one myeloid cell lineage in the bone marrow. It primarily affects older adults and includes refractory anemia, refractory neutropenia, and refractory thrombocytopenia. Malignant C97310 Myelodysplastic Syndrome with Somatic Mutations Myelodysplastic Syndrome with Point Mutations A myelodysplastic syndrome characterized by the presence of a point mutation in one of the following genes, TP53, EZH2, ETV6, RUNX1, and ASXL1. These gene mutations are independent predictors of poor prognosis. Malignant C8648 Myelodysplastic Syndrome, Unclassifiable Unclassifiable MDS || Unclassifiable Myelodysplastic Syndrome A subtype of myelodysplastic syndrome which initially lacks findings appropriate for classification into any other myelodysplastic category. There are no specific morphologic findings. The diagnosis can be made in the following instances: 1. in cases of refractory cytopenia with unilineage dysplasia or refractory cytopenia with multilineage dysplasia but with 1% blasts in the peripheral blood; 2: in cases of myelodysplastic syndrome with unilineage dysplasia which are associated with pancytopenia; 3: in cases with persistent cytopenia (s) with 1% or fewer blasts in the blood and fewer than 5% in the bone marrow, unequivocal dysplasia in less than 10% of the cells in one or more myeloid lineages, and cytogenetic abnormalities considered as presumptive evidence of myelodysplastic syndrome. (WHO, 2008) Malignant C27262 Myelodysplastic/Myeloproliferative Neoplasm Myelodysplastic/Myeloproliferative Disease || Myelodysplastic/Myeloproliferative Diseases || Myelodysplastic/Myeloproliferative Disorder || Myelodysplastic/Myeloproliferative Disorders || Myeloproliferative/Myelodysplastic Disorders || Myeloproliferative/Myelodysplastic Syndromes A category of clonal hematopoietic disorders that have both myelodysplastic and myeloproliferative features at the time of initial presentation. Malignant C82616 Myelodysplastic/Myeloproliferative Neoplasm with Ring Sideroblasts and Thrombocytosis Essential Thrombocythemia with Ring Sideroblasts || MDS/MPN with Ring Sideroblasts and Thrombocytosis || Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis A myelodysplastic/myeloproliferative neoplasm with morphologic and clinical characteristics of refractory anemia with ring sideroblasts, marked thrombocytosis, and abnormal megakaryocytes. Malignant C27780 Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable Mixed Myelodysplastic/Myeloproliferative Disease, Unclassifiable || Mixed Myeloproliferative/Myelodysplastic Syndrome, Unclassifiable || Myelodysplastic/Myeloproliferative Disease, Unclassifiable || Unclassifiable Myelodysplastic/Myeloproliferative Disease || Unclassifiable Myeloproliferative/Myelodysplastic Syndrome This entity includes cases that have clinical, laboratory, and morphologic features that support the diagnosis of both a myelodysplastic syndrome and a myeloproliferative neoplasm, but do not meet the criteria for any of the other entities included in the myelodysplastic/myeloproliferative neoplasm category. (WHO, 2001) Malignant C43223 Myeloid Leukemia Associated with Down Syndrome Acute myeloid leukemia or myelodysplastic syndrome occurring in children with Down syndrome. The acute myeloid leukemia is usually an acute megakaryoblastic leukemia, and is associated with GATA1 gene mutation. Malignant C82338 Myeloid Proliferations Associated with Down Syndrome Myeloid Proliferations Related to Down Syndrome Myeloid neoplasms occurring in individuals with Down syndrome. There is an increased risk of acute leukemias in both children and adults with Down syndrome. In particular, the incidence of acute myeloid leukemia in Down syndrome children of less than five years of age is particularly high, it is usually an acute megakaryoblastic leukemia, and is associated with GATA1 gene mutation. This group of disorders also includes the entity transient abnormal myelopoiesis which occurs in neonates and is associated with GATA1 gene mutation. Malignant C3520 Myeloid Sarcoma Chloroma || Extramedullary Myeloid Tumor A tumor mass composed of myeloblasts or immature myeloid cells. It occurs in extramedullary sites or the bone. (WHO, 2001) Malignant C84270 Myeloid/Lymphoid Neoplasms with Eosinophilia and Rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2 Myeloid and Lymphoid Neoplasms with Eosinophilia and Abnormalities of PDGFRA, PDGFRB, or FGFR1 || Myeloid and Lymphoid Neoplasms with Eosinophilia and Rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2 || Myeloid/Lymphoid Neoplasms with Eosinophilia and Gene Rearrangement || Myeloid/Lymphoid Neoplasms with Eosinophilia and Rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2 A group of rare myeloid and lymphoid neoplasms characterized by rearrangement of the PDGFRA, PDGFRB, or FGFR1 genes, resulting in the formation of fusion transcripts and aberrant tyrosine kinase activity. Eosinophilia is a characteristic finding but it is not always present. This group includes the myeloid and lymphoid neoplasms with t(8;9)(p22;p24.1);PCM1-JAK2 as a provisional entity. Malignant C84277 Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement 8p11 Myeloproliferative Syndrome || 8p11 Stem Cell Leukemia/Lymphoma Syndrome || 8p11 Stem Cell Lymphoma/Leukemia Syndrome || 8p11 Stem Cell Syndrome || Myeloid and Lymphoid Neoplasms with FGFR1 Rearrangement Hematologic neoplasms characterized by the rearrangement of the FGFR1 gene, resulting in translocations with an 8p11 breakpoint. Patients present with a myeloproliferative neoplasm, acute myeloid leukemia, lymphoblastic lymphoma/leukemia of T or B-cell lineage, or acute leukemia of mixed phenotype. Malignant C84275 Myeloid/Lymphoid Neoplasms with PDGFRA Rearrangement Myeloid and Lymphoid Neoplasms with PDGFRA Rearrangement Hematologic neoplasms characterized by the rearrangement of the PDGFRA gene, most often resulting in the formation of FIP1L1-PDGFRA fusion transcripts. Patients usually present with chronic eosinophilic leukemia, and less often with acute myeloid leukemia or T-lymphoblastic leukemia. Malignant C84276 Myeloid/Lymphoid Neoplasms with PDGFRB Rearrangement Myeloid Neoplasms with PDGFRB Rearrangement || Myeloid and Lymphoid Neoplasms with PDGFRB Rearrangement Myeloid and rarely lymphoid neoplasms characterized by the rearrangement of the PDGFRB gene, most often resulting in the formation of ETV6-PDGFRB fusion transcripts. Patients usually present with chronic myelomonocytic leukemia and less often with atypical chronic myeloid leukemia, or chronic eosinophilic leukemia. Malignant C4345 Myeloproliferative Neoplasm Chronic Myeloproliferative Disorder || Chronic Myeloproliferative Neoplasm || Myeloproliferative Disease || Myeloproliferative Disorder || Myeloproliferative Tumor A clonal hematopoietic stem cell disorder, characterized by proliferation in the bone marrow of one or more of the myeloid (i.e., granulocytic, erythroid, megakaryocytic, and mast cell) lineages. It is primarily a neoplasm of adults. (WHO 2008) Malignant C27350 Myeloproliferative Neoplasm, Unclassifiable Chronic Myeloproliferative Disease, Unclassifiable || Chronic Myeloproliferative Disorder, Unclassifiable || Unclassifiable Chronic Myeloproliferative Disease || Unclassifiable Chronic Myeloproliferative Disorder This subgroup of myeloproliferative neoplasms includes cases which do not meet the morphologic criteria of any of the defined myeloproliferative neoplasms, or which have characteristics that overlap at least two of the myeloproliferative neoplasms. Malignant C3701 Myxoid Leiomyosarcoma A morphologic variant of leiomyosarcoma characterized by the presence of cellular pleomorphism, malignant cells with large nuclei, and a myxoid stroma. Malignant C27781 Myxoid Liposarcoma Myxoid Liposarcoma || Myxoid/Round Cell Liposarcoma A liposarcoma characterized by the presence of round non-lipogenic primitive mesenchymal cells and small signet ring lipoblasts within a myxoid stoma with a branching vascular pattern. This category includes hypercellular lesions with round cell morphology, formerly known as round cell liposarcoma. Malignant C3697 Myxopapillary Ependymoma A slow growing, WHO grade I glioma which generally occurs in young adults. It arises almost exclusively in the conus medullaris, cauda equina, and filum terminale of the spinal cord. It generally has a favorable prognosis and is characterized histologically by tumor cells arranged in a papillary manner around vascularized mucoid stromal cores. (Adapted from WHO). Undetermined C8192 Nasal Cavity Squamous Cell Carcinoma Squamous Cell Carcinoma of Nasal Cavity || Squamous Cell Carcinoma of the Nasal Cavity A squamous cell carcinoma that arises from the nasal cavity mucosa. Signs and symptoms include nasal fullness and obstruction, pain, epistaxis, and the presence of a nasal mass. Malignant C4684 Nasal Type Extranodal NK/T-Cell Lymphoma Angiocentric T-Cell Lymphoma || Extranodal NK/T-Cell Lymphoma, Nasal Type An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterized by an often angiocentric and angiodestructive cellular infiltrate composed of EBV positive NK/T cells. The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell lymphoma. The term "polymorphic reticulosis" has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder. Malignant C7992 Nasopharyngeal Keratinizing Squamous Cell Carcinoma Keratinizing Epidermoid Carcinoma of Nasopharynx || Keratinizing Epidermoid Carcinoma of the Nasopharynx || Keratinizing Squamous Cell Carcinoma of Nasopharynx || Keratinizing Squamous Cell Carcinoma of the Nasopharynx || Nasopharyngeal Keratinizing Epidermoid Carcinoma A squamous cell carcinoma that arises from the nasopharynx and is characterized by prominent production of keratin. Malignant C6077 Neck Carcinoma Carcinoma of Neck || Carcinoma of the Neck A carcinoma that arises from the anatomic structures of the neck region. Malignant C7232 Neoplastic Post-Transplant Lymphoproliferative Disorder Neoplastic PTLD || Post-Transplant Lymphocytic Neoplasm || Post-Transplant Lymphoproliferative Neoplasm || Post-Transplant Neoplastic Lymphoproliferative Disorder A lymphoproliferative disorder arising as a result of post-transplant immunosuppression therapy. It is characterized by the presence of tissue destruction and effacement of the architecture of the involved tissues. There is clonal rearrangement of the immunoglobulin or the T-cell receptor genes. It includes the following variants: polymorphic, monomorphic, and classical Hodgkin lymphoma type post-transplant lymphoproliferative disorders. Undetermined C7018 Nerve Sheath Myxoma Neurothekeoma A benign neoplasm arising from nerve sheaths. It is characterized by the presence of a myxoid stroma. Benign C111693 Neural Glioblastoma A molecular subtype of glioblastoma characterized by the expression of the neural markers NEFL, GABRA1, SYT1, and SLC12A5. Malignant C3270 Neuroblastoma Neuroblastoma (Schwannian Stroma-Poor) A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation. Malignant C3773 Neuroendocrine Carcinoma Neuroendocrine Carcinoma A usually aggressive carcinoma composed of malignant cells exhibiting neuroendocrine differentiation. Malignant C3809 Neuroendocrine Neoplasm A benign or malignant neoplasm composed of cells of neuroendocrine origin. Representative examples include paraganglioma, carcinoid tumor, and neuroendocrine carcinoma. Undetermined C3272 Neurofibroma Neurofibroma (WHO Grade I) An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Patients with neurofibromatosis type 1 present with multiple masses. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors. Benign C4192 Nipple Adenoma Adenoma of Nipple || Adenoma of the Nipple A benign epithelial neoplasm arising from the nipple. Signs and symptoms include serous or sanguineous nipple discharge and nipple erosion. It is characterized by the presence of aggregates of small tubules replacing the nipple stroma. The tubules are lined by epithelial and myoepithelial cells. Benign C28432 Nipple Carcinoma A carcinoma that arises in the area of the nipple. Representative examples include Paget disease and skin squamous cell carcinoma. Malignant C8863 Nodal Marginal Zone Lymphoma Monocytoid B-Cell Lymphoma || Nodal Marginal Zone B-Cell Lymphoma A primary nodal B-cell non-Hodgkin lymphoma which morphologically resembles lymph nodes involved by marginal zone lymphomas of extranodal or splenic types, but without evidence of extranodal or splenic disease. (WHO, 2001) Malignant C7258 Nodular Lymphocyte Predominant Hodgkin Lymphoma Nodular Lymphocyte Predominant Hodgkin's Lymphoma A monoclonal B-cell neoplasm characterized by a nodular, or a nodular and diffuse proliferation of scattered large neoplastic cells known as popcorn or lymphocyte predominant cells (LP cells)- formerly called L&H cells for lymphocytic and/or histiocytic Reed-Sternberg cell variants. The LP cells lack CD15 and CD30 in nearly all instances. Patients are predominantly male, frequently in the 30-50 year age group. Most patients present with limited stage disease (localized peripheral lymphadenopathy, stage I or II). (WHO 2008) Malignant C3518 Nodular Sclerosis Classic Hodgkin Lymphoma Hodgkin's Nodular Sclerosis || Nodular Sclerosis Hodgkin Lymphoma || Nodular Sclerosis Hodgkin's Disease || Nodular Sclerosis Hodgkin's Lymphoma A subtype of classic Hodgkin lymphoma characterized by collagen bands that surround at least one nodule, and Hodgkin and Reed-Sternberg cells with lacunar type morphology. (WHO, 2008) Malignant C6951 Nonanaplastic Kidney Wilms Tumor Nonanaplastic Renal Wilm's Tumor || Nonanaplastic Renal Wilms Tumor || Nonanaplastic Renal Wilms' Tumor Wilms tumor of the kidney characterized by the absence of nuclear anaplasia. Malignant C8711 Non-Cutaneous Melanoma Extracutaneous Melanoma A melanoma that arises from a site other than skin. Malignant C4348 Non-Functioning Pituitary Gland Adenoma Functionless Adenoma of Pituitary || Functionless Adenoma of Pituitary Gland || Functionless Adenoma of the Pituitary || Functionless Adenoma of the Pituitary Gland || Functionless Pituitary Adenoma || Functionless Pituitary Gland Adenoma || Non-Functioning Adenoma of Pituitary || Non-Functioning Adenoma of Pituitary Gland || Non-Functioning Adenoma of the Pituitary || Non-Functioning Adenoma of the Pituitary Gland || Non-Functioning Pituitary Adenoma || Non-Secretory Adenoma of Pituitary || Non-Secretory Adenoma of Pituitary Gland || Non-Secretory Adenoma of the Pituitary || Non-Secretory Adenoma of the Pituitary Gland || Non-Secretory Pituitary Adenoma || Non-Secretory Pituitary Gland Adenoma || Nonfunctional Pituitary Gland Adenoma || Nonfunctioning Pituitary Gland Adenoma || Silent Pituitary Gland Adenoma A hormone producing or non-producing pituitary gland adenoma not associated with a hormonal syndrome. Benign C39991 Non-Gestational Ovarian Choriocarcinoma A malignant germ cell tumor that arises from the ovary and is composed of cytotrophoblasts, syncytiotrophoblasts, and extravillous trophoblasts. The prognosis is less favorable than gestational choriocarcinoma. Malignant C36261 Non-Hereditary Clear Cell Renal Cell Carcinoma Non-Hereditary Conventional (Clear Cell) Renal Cell Carcinoma || Sporadic Clear Cell Renal Cell Carcinoma || Sporadic Conventional (Clear Cell) Renal Cell Carcinoma The most common type of renal cell carcinoma, characterized by a loss of genetic material of the short arm of chromosome 3. The most common symptom at the time of diagnosis is hematuria. The tumor has a golden-yellow color because of the abundance of intracytoplasmic lipid. This is a clinically aggressive type of renal cell carcinoma. The tumor usually metastasizes to unusual sites and late metastasis is common. Malignant C3211 Non-Hodgkin Lymphoma Non-Hodgkin's Lymphoma || Non-Hodgkin's Lymphoma (NHL) Distinct from Hodgkin lymphoma both morphologically and biologically, non-Hodgkin lymphoma (NHL) is characterized by the absence of Reed-Sternberg cells, can occur at any age, and usually presents as a localized or generalized lymphadenopathy associated with fever and weight loss. The clinical course varies according to the morphologic type. NHL is clinically classified as indolent, aggressive, or having a variable clinical course. NHL can be of B-or T-/NK-cell lineage. Malignant C9080 Noninvasive Malignant Thymoma Malignant Thymoma, Noninvasive || Thymoma Malignant Noninvasive A morphologically malignant thymoma that is entirely confined within the capsule. Malignant C4734 Non-Secretory Plasma Cell Myeloma Non-Secretory Multiple Myeloma A rare type of multiple myeloma in which the plasma cells synthesize but do not secrete immunoglobulins. As a result, none of the immunoglobulins appear out of the normal range. The symptoms are generally the same with those of immunoglobulin-secreting myeloma; however, the incidence of renal insufficiency is lower in non-secretory myeloma. The diagnosis can be missed because of the absence of monoclonal immunoglobulin in the serum or urine. Malignant C53557 Normal Breast-Like Subtype of Breast Carcinoma Normal Breast-Like Subtype of Breast Cancer A biologic subset of breast carcinoma defined by high expression of many genes expressed by adipose and other non-epithelial tissues. Malignant C45924 Null Cell Pituitary Gland Adenoma Hormone Immunonegative Adenoma || Hormone Immunonegative Pituitary Gland Adenoma || Null Cell Adenoma || Transcription Factor-Immunonegative Adenoma A non-functioning pituitary gland adenoma without hormonal immunoreactivity or evidence of specific adenohypophysial cell derivation. Benign C45716 NUT Carcinoma Carcinoma with t(15;19)(q13;p13.1) Translocation || Midline Carcinoma of Children and Young Adults with NUT Rearrangement || NUT Midline Carcinoma || Nuclear Protein in Testis (NUT) Midline Carcinoma || Nuclear Protein in Testis Midline Carcinoma A rare, highly aggressive and lethal carcinoma that affects children and young adults. It arises from midline epithelial structures, most commonly the head, neck, and mediastinum. It is a poorly differentiated carcinoma and is characterized by mutations and rearrangement of the NUT gene. A balanced translocation t(15;19) is present that results in the creation of a fusion gene involving the NUT gene, most commonly BRD4-NUT fusion gene. Malignant C35689 Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Ocular Adnexal MALT lymphoma An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the ocular adnexa. It is the most common type of primary ocular adnexal lymphoma. Malignant C3789 Olfactory Neuroblastoma Esthesioneuroblastoma || Esthesioneuroepithelioma || Olfactory Esthesioneuroblastoma || Olfactory Neuroepithelioma A rare neuroectodermal tumor originating from olfactory receptor cells in the nasal cavity or paranasal sinuses. Microscopically, it is characterized by neuroblastic differentiation with occasional formation of rosettes. If the tumor is not resected at an early stage, the prognosis is usually poor. Malignant C4050 Oligoastrocytoma Mixed Astrocytic-Oligodendroglial Neoplasm || Mixed Astrocytic-Oligodendroglial Tumor || Mixed Astrocytoma-Oligodendroglioma || Mixed Oligo-Astrocytoma || WHO Grade II Mixed Glioma A WHO grade II tumor composed of a conspicuous mixture of two distinct neoplastic cell types morphologically resembling the tumor cells in oligodendroglioma and diffuse astrocytoma. (WHO) Undetermined C6960 Oligodendroglial Tumor Oligodendroglial Neoplasm A neoplasm of the central nervous system composed predominantly of cells morphologically resembling oligodendroglia. Oligodendroglial tumors histologically comprise a continuous spectrum ranging from well-differentiated neoplasms to frankly malignant tumors. WHO grading system recognizes two malignancy grades: WHO grade II for well-differentiated tumors (oligodendroglioma) and WHO grade III for anaplastic oligodendroglioma. (Adapted from WHO) Undetermined C3288 Oligodendroglioma WHO Grade II Oligodendroglial Neoplasm || WHO Grade II Oligodendroglial Tumor || Well Differentiated Oligodendroglial Tumor || Well Differentiated Oligodendroglioma A well-differentiated (WHO grade II), diffusely infiltrating neuroglial tumor, typically located in the cerebral hemispheres. It is composed predominantly of cells which morphologically resemble oligodendroglia. The neoplastic cells have rounded homogeneous nuclei and, on paraffin sections, a swollen, clear cytoplasm ('honeycomb' appearance). (Adapted from WHO) Undetermined C129318 Oligodendroglioma, IDH-Mutant and 1p/19q-Codeleted An oligodendroglioma carrying IDH gene family mutation and combined whole-arm losses of 1p and 19q (1p/19q codeletion). Undetermined C129319 Oligodendroglioma, Not Otherwise Specified Oligodendroglioma, NOS An oligodendroglioma in which there is insufficient information on the IDH genes and 1p/19q codeletion status. Undetermined C3679 Oncocytic Adenocarcinoma Hurthle Cell Adenocarcinoma || Hurthle Cell Carcinoma || Oncocytic Carcinoma || Oxyphilic Adenocarcinoma An adenocarcinoma characterized by the presence of large malignant epithelial cells with abundant granular eosinophilic cytoplasm (oncocytes). Representative examples include thyroid gland oncocytic follicular carcinoma, oncocytic breast carcinoma, and salivary gland oncocytic carcinoma. Malignant C8990 Oral Cavity Carcinoma Carcinoma of Mouth || Carcinoma of Oral Cavity || Carcinoma of the Oral Cavity || Mouth Carcinoma A carcinoma arising in the oral cavity. Most oral cavity carcinomas are squamous cell carcinomas of the tongue, buccal mucosa, or gums. Less frequent morphologic variants include mucoepidermoid carcinoma and adenocarcinoma. Malignant C6242 Oral Cavity Pleomorphic Adenoma Benign Mixed Salivary Tumor of Oral Cavity || Benign Mixed Salivary Tumor of the Oral Cavity || Pleomorphic Adenoma of Oral Cavity || Pleomorphic Adenoma of the Oral Cavity A benign, slow-growing and painless neoplasm that arises from the salivary glands in the oral cavity. It is composed of cells that demonstrate both epithelial and mesenchymal differentiation. Benign C4833 Oral Cavity Squamous Cell Carcinoma Mouth SCC || Mouth Squamous Cell Carcinoma || Oral Cavity SCC || SCC of Mouth || SCC of Oral Cavity || SCC of the Mouth || SCC of the Oral Cavity || Squamous Cell Carcinoma of Mouth || Squamous Cell Carcinoma of Oral Cavity || Squamous Cell Carcinoma of the Mouth || Squamous Cell Carcinoma of the Oral Cavity A squamous cell carcinoma arising from the oral cavity. It affects predominantly adults in their fifth and sixth decades of life and is associated with alcohol and tobacco use. Human papillomavirus is present in approximately half of the cases. It is characterized by a tendency to metastasize early to the lymph nodes. When the tumor is small, patients are often asymptomatic. Physical examination may reveal erythematous or white lesions or plaques. The majority of patients present with signs and symptoms of locally advanced disease including mucosal ulceration, pain, difficulty with speaking, chewing, and swallowing, bleeding, weight loss, and neck swelling. Patients may also present with swollen neck lymph nodes without any symptoms from the oropharyngeal tumor. The most significant prognostic factors are the size of the tumor and the lymph nodes status. Malignant C8181 Oropharyngeal Squamous Cell Carcinoma Epidermoid Carcinoma of Oropharynx || Epidermoid Carcinoma of the Oropharynx || Oropharyngeal Epidermoid Carcinoma || Squamous Cell Carcinoma of Oropharynx || Squamous Cell Carcinoma of the Oropharynx A squamous cell carcinoma arising from the oropharynx. It predominantly affects adults in their fifth and sixth decades of life and is associated with alcohol and tobacco use. Human papillomavirus is present in approximately half of the cases. It is characterized by a tendency to metastasize early to the lymph nodes. When the tumor is small, patients are often asymptomatic. Physical examination may reveal erythematous or white lesions or plaques. The majority of patients present with locally advanced disease. Signs and symptoms include mucosal ulceration, pain, bleeding, weight loss, neck swelling, and difficulty speaking, chewing, and swallowing. Patients may also present with swollen neck lymph nodes without any symptoms from the oropharyngeal tumor. The most significant prognostic factors are the size of the tumor and the lymph nodes status. Malignant C53953 Osteoblastic Osteosarcoma A conventional osteosarcoma characterized by the predominance of osteoid matrix. Malignant C3294 Osteoblastoma Giant Osteoid Osteoma || Ossifying Giant Cell Tumor A rare benign bone-forming neoplasm usually arising from the spine. It is a well-circumscribed lytic tumor that varies in size. The tumor is composed of woven bone trabeculae and shares similar histologic characteristics with the osteoid osteoma. Surgical curettage is the treatment of choice. The prognosis is excellent. Benign C3738 Osteoclastic Giant Cell-Rich Tumor of Bone Osteoclastic Giant Cell-Rich Tumor of the Bone A benign, intermediate, or malignant bone tumor characterized by the presence of osteoclast-like giant cells. Clinical presentation includes pain, edema, and decreased range of motion in the affected joint. Undetermined C3297 Osteoid Osteoma A small, benign, bone-forming neoplasm that can arise from any bone but more frequently affects the long bones. The central portion of the neoplasm (nidus) contains differentiated osteoblasts which produce osteoid and sometimes bone. The lesion is usually surrounded by hypervascular sclerotic bone and has limited growth potential. Clinical signs and symptoms include pain and localized tenderness, at the site of the lesion. The pain may be intense but in the majority of cases it is completely alleviated by non-steroidal anti-inflammatory drugs. Prognosis is excellent and recurrences are rare. Benign C3296 Osteoma A benign, well-circumscribed, bone-forming neoplasm predominantly composed of lamellar bone. It usually arises from the calvarial, facial, or jaw bones. It is usually asymptomatic but it may cause local swelling or obstruction of the paranasal sinuses. Asymptomatic cases have an indolent clinical course. Benign C9145 Osteosarcoma Osteogenic Sarcoma A usually aggressive malignant bone-forming mesenchymal neoplasm, predominantly affecting adolescents and young adults. It usually involves bones and less frequently extraosseous sites. It often involves the long bones (particularly distal femur, proximal tibia, and proximal humerus). Pain with or without a palpable mass is the most frequent clinical symptom. It may spread to other anatomic sites, particularly the lungs. Malignant C6469 Osteosarcoma Arising in Paget Disease of Bone Osteosarcoma Arising in Bone Paget's Disease || Osteosarcoma Arising in Osseous Paget's Disease || Osteosarcoma Arising in Osteitis Deformans || Osteosarcoma Arising in Paget's Disease of Bone || Paget Osteosarcoma || Paget's Osteosarcoma A sarcomatous transformation of pre-existing Paget disease of the bone. Osteosarcomas arising from Paget disease of the bone are high grade lesions and usually have a poor prognosis. Malignant C7765 Osteosclerotic Plasma Cell Myeloma Osteosclerotic Multiple Myeloma A plasma cell neoplasm associated with osteosclerotic and fibrotic changes in the bone trabeculae. Often, the lymph nodes show changes resembling the plasma cell variant of Castleman disease. It is often part of POEMS syndrome which is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Malignant C7700 Ovarian Adenocarcinoma Adenocarcinoma of Ovary || Adenocarcinoma of the Ovary An adenocarcinoma that arises from the ovary. It is the most common type of ovarian carcinoma. It includes the serous adenocarcinoma, mucinous adenocarcinoma, clear cell adenocarcinoma, and endometrioid adenocarcinoma. Malignant C4908 Ovarian Carcinoma Carcinoma of Ovary || Carcinoma of the Ovary A malignant neoplasm originating from the surface ovarian epithelium. It accounts for the greatest number of deaths from malignancies of the female genital tract and is the fifth leading cause of cancer fatalities in women. It is predominantly a disease of older white women of northern European extraction, but it is seen in all ages and ethnic groups. Adenocarcinomas constitute the vast majority of ovarian carcinomas. The pattern of metastatic spread in ovarian carcinoma is similar regardless of the microscopic type. The most common sites of involvement are the contralateral ovary, peritoneal cavity, para-aortic and pelvic lymph nodes, and liver. Lung and pleura are the most common sites of extra-abdominal spread. The primary form of therapy is surgical. The overall prognosis of ovarian carcinoma remains poor, a direct result of its rapid growth rate and the lack of early symptoms. Malignant C40078 Ovarian Clear Cell Adenocarcinoma A malignant glandular epithelial neoplasm characterized by the presence of clear and hobnail cells. It is highly associated with ovarian endometriosis, pelvic endometriosis and paraendocrine hypercalcemia. Malignant C5228 Ovarian Cystadenocarcinoma Cystadenocarcinoma of Ovary || Cystadenocarcinoma of the Ovary An adenocarcinoma that arises from the ovary and is characterized by the presence of cystic structures. It includes the serous cystadenocarcinoma, mucinous cystadenocarcinoma, and clear cell cystadenocarcinoma. Malignant C8106 Ovarian Dysgerminoma Dysgerminoma of Ovary || Dysgerminoma of the Ovary || Germ Cell Dysgerminoma of Ovary || Germ Cell Dysgerminoma of the Ovary || Ovarian Germ Cell Dysgerminoma A malignant germ cell tumor arising from the ovary. Morphologically, it is identical to seminoma and consists of a monotonous population of germ cells with abundant pale cytoplasm and uniform nuclei. The stroma invariably contains chronic inflammatory cells, mostly T-lymphocytes. It responds to chemotherapy or radiotherapy and the prognosis relates to the tumor stage. Malignant C7979 Ovarian Endometrioid Adenocarcinoma Endometrioid Adenocarcinoma of Ovary || Endometrioid Adenocarcinoma of the Ovary || Endometrioid Cancer of Ovary || Endometrioid Cancer of the Ovary || Endometrioid Carcinoma of Ovary || Endometrioid Carcinoma of the Ovary || Ovarian Endometrioid Adenocarcinoma NOS || Ovarian Endometrioid Adenocarcinoma Not Otherwise Specified || Ovarian Endometrioid Cancer || Ovarian Endometrioid Carcinoma An endometrioid adenocarcinoma arising from the ovary. It comprises 10% to 25% of all primary ovarian carcinomas. Grossly, endometrioid carcinoma may present as a cystic or solid mass. Microscopically, the tumor greatly resembles the appearance of the ordinary type of endometrial adenocarcinoma. As a group, endometrioid carcinoma has a prognosis twice as good as that of serous or mucinous carcinoma. Malignant C3873 Ovarian Germ Cell Tumor Germ Cell Neoplasm of Ovary || Germ Cell Neoplasm of the Ovary || Germ Cell Tumor of Ovary || Germ Cell Tumor of the Ovary || Ovarian Germ Cell Neoplasm A neoplasm that arises from the ovary and originates from germ cells. Representative examples include teratoma, embryonal carcinoma, yolk sac tumor, and dysgerminoma. Undetermined C5243 Ovarian Mucinous Adenocarcinoma Mucinous Adenocarcinoma of Ovary || Mucinous Adenocarcinoma of the Ovary || Mucinous Carcinoma of Ovary || Mucinous Carcinoma of the Ovary || Ovarian Mucinous Carcinoma An invasive adenocarcinoma that arises from the ovary and is characterized by the presence of malignant epithelial cells that contain intracytoplasmic mucin. There is cellular atypia, increased layering of cells, complexity of glands, and papillary formations. Malignant C4512 Ovarian Mucinous Cystadenoma Benign Mucinous Cystadenoma of Ovary || Benign Mucinous Cystadenoma of the Ovary || Benign Ovarian Mucinous Cystadenoma || Mucinous Cystadenoma of Ovary || Mucinous Cystadenoma of the Ovary A benign neoplasm of the ovary characterized by the presence of cystic structures lined by mucinous columnar epithelial cells. Benign C7550 Ovarian Serous Adenocarcinoma Ovarian Serous Carcinoma || Serous Adenocarcinoma of Ovary || Serous Adenocarcinoma of the Ovary || Serous Carcinoma of Ovary || Serous Carcinoma of the Ovary An adenocarcinoma that arises from the ovary and is characterized by the presence of malignant epithelial cells that, in well differentiated tumors, resemble the epithelium of the fallopian tube or, in poorly differentiated tumors, show anaplastic features and marked nuclear atypia. Malignant C4511 Ovarian Serous Cystadenoma Benign Ovarian Serous Cystadenoma || Benign Serous Cystadenoma of Ovary || Benign Serous Cystadenoma of the Ovary || Serous Cystadenoma of Ovary || Serous Cystadenoma of the Ovary A benign, usually bilateral neoplasm of the ovary characterized by the presence of cystic structures that contain watery fluid and are lined by serous epithelial cells. Signs and symptoms include abdominal enlargement, pain, and vaginal bleeding. Benign C27390 Ovarian Small Cell Carcinoma A carcinoma that arises from the ovary and is characterized by the presence of small malignant cells. It includes small cell carcinoma, hypercalcemic type and small cell carcinoma, pulmonary type. Malignant C40093 Ovarian Squamous Cell Carcinoma A usually high grade squamous cell carcinoma that arises from the ovary and is not associated with a germ cell tumor. The prognosis is poor. Undetermined || Malignant C5240 Ovarian Transitional Cell Carcinoma Transitional Cell Carcinoma of Ovary || Transitional Cell Carcinoma of the Ovary A carcinoma that arises from the ovary and is characterized by the presence of malignant epithelial cells that resemble malignant urothelial cells. Malignant C4509 Ovarian Undifferentiated Carcinoma Anaplastic Carcinoma of Ovary || Anaplastic Carcinoma of the Ovary || Anaplastic Ovarian Carcinoma || Undifferentiated Carcinoma of Ovary || Undifferentiated Carcinoma of the Ovary || Undifferentiated Ovarian Carcinoma An aggressive carcinoma arising from the ovary. Most patients present with advanced disease. Microscopically, it is characterized by significant cytologic atypia, increased mitotic activity, and necrosis. The prognosis is usually poor. Malignant C41238 Overt Primary Myelofibrosis Chronic Idiopathic Myelofibrosis, Fibrotic Stage || Primary Myelofibrosis, Fibrotic Stage || Primary Myelofibrosis, Overt Fibrotic Stage Primary myelofibrosis characterized by reticulin or collagen fibrosis in the bone marrow. The bone marrow is usually normocellular or hypocellular. Myeloblasts account for less than 10% of the bone marrow cells. Atypical megakaryocytes are present. Malignant C7073 Paget Disease Paget Cell Neoplasm || Paget's Cell Neoplasm || Paget's Disease A malignant neoplasm composed of large cells with large nuclei, prominent nucleoli, and abundant pale cytoplasm (Paget cells). Paget cell neoplasms include Paget disease of the nipple and extramammary Paget disease which may affect the vulva, penis, anus, skin and scrotum. Malignant C4649 Palate Squamous Cell Carcinoma Palate SCC || SCC of Palate || SCC of the Palate || Squamous Cell Carcinoma of Palate || Squamous Cell Carcinoma of the Palate A squamous cell carcinoma that arises from the hard or soft palate. Malignant C7977 Pancreatic Acinar Cell Carcinoma Acinar Cell Adenocarcinoma of Pancreas || Acinar Cell Adenocarcinoma of the Pancreas || Acinar Cell Carcinoma of Pancreas || Acinar Cell Carcinoma of the Pancreas || Pancreas Acinar Cell Adenocarcinoma || Pancreatic Acinar Cell Adenocarcinoma An adenocarcinoma arising from the pancreas. It is characterized by the presence of relatively uniform malignant cells which form acinar patterns. It usually occurs during adulthood. Signs and symptoms include abdominal pain, weight loss, nausea, and diarrhea. It may metastasize to regional lymph nodes and the liver. A minority of patients develop lipase hypersecretion syndrome. This syndrome may be seen in patients with liver metastases and it is characterized by excessive secretion of lipase in the serum, polyarthralgia, and subcutaneous fat necrosis. Malignant C8294 Pancreatic Adenocarcinoma Adenocarcinoma of Pancreas || Adenocarcinoma of the Pancreas || Pancreas Adenocarcinoma An adenocarcinoma which arises from the exocrine pancreas. Ductal adenocarcinoma and its variants are the most common types of pancreatic adenocarcinoma. Malignant C3850 Pancreatic Carcinoma Cancer of Pancreas || Cancer of the Pancreas || Carcinoma of Pancreas || Carcinoma of the Pancreas || Exocrine Pancreas Carcinoma || Pancreas Cancer || Pancreas Carcinoma || Pancreatic Cancer A carcinoma arising from the exocrine pancreas. The overwhelming majority of pancreatic carcinomas are adenocarcinomas. Malignant C3874 Pancreatic Cystadenocarcinoma Cystadenocarcinoma of Pancreas || Cystadenocarcinoma of the Pancreas A cystic adenocarcinoma that arises from the pancreas. It includes the acinar cell and serous cystadenocarcinoma subtypes. Malignant C4374 Pancreatic Cystadenoma Cystadenoma of Pancreas || Cystadenoma of the Pancreas A non-metastasizing cystic epithelial neoplasm arising from the exocrine pancreas. Benign C28396 Pancreatic Delta Cell Neuroendocrine Tumor Pancreatic Delta Cell NET || Pancreatic Delta Cell Neoplasm || Pancreatic Delta Cell Tumor A usually malignant neuroendocrine tumor arising from the delta cells of the pancreas. It may be associated with inappropriate secretion of somatostatin and an associated clinical syndrome, or it may be hormonally inactive (non-functioning). Undetermined C9120 Pancreatic Ductal Adenocarcinoma Ductal Adenocarcinoma of Pancreas || Ductal Adenocarcinoma of the Pancreas || Pancreas Ductal Adenocarcinoma || Pancreatic Infiltrating Duct Carcinoma, NOS || Pancreatic Infiltrating Duct Carcinoma, Not Otherwise Specified || Pancreatic Tubular Adenocarcinoma An infiltrating adenocarcinoma that arises from the epithelial cells of the pancreas. It affects males more often than females and the patients are usually over 50 years of age. Microscopically it is characterized by the presence of glandular (ductal) differentiation and desmoplastic stroma formation. Signs and symptoms include pain, loss of weight, and jaundice. It grows rapidly and is usually detected after it has metastasized to other anatomic sites. The prognosis is usually poor. Malignant C9069 Pancreatic Gastrin-Producing Neuroendocrine Tumor Pancreatic Gastrin Producing NET || Pancreatic Gastrin Producing Tumor A usually malignant gastrin-producing neuroendocrine tumor arising from the pancreas. It may or may not be associated with inappropriate secretion of gastrin and an associated clinical syndrome. Undetermined C3062 Pancreatic Glucagon-Producing Neuroendocrine Tumor Alpha Cell Tumor || Alpha Cell Tumor of Pancreas || Alpha Cell Tumor of the Pancreas || Glucagon-Producing Islet Cell Tumor || Glucagon-Producing Tumor of Islet Cells || Glucagon-Producing Tumor of the Islet Cells || Pancreatic Alpha Cell Tumor || Pancreatic Glucagon Producing NET || Pancreatic Glucagon Producing Neuroendocrine Tumor || Pancreatic Glucagon Producing Tumor A usually malignant, glucagon-producing neuroendocrine tumor arising from the pancreatic alpha cells. It may or may not be associated with inappropriate secretion of glucagon and an associated clinical syndrome. Undetermined C3140 Pancreatic Insulin-Producing Neuroendocrine Tumor Beta Cell Tumor || Beta Cell Tumor of Pancreas || Beta Cell Tumor of the Pancreas || Insulin-Producing Islet Cell Tumor || Insulin-Producing Tumor of Islet Cells || Insulin-Producing Tumor of the Islet Cells || Pancreatic Beta Cell Tumor || Pancreatic Insulin Producing NET || Pancreatic Insulin Producing Neoplasm || Pancreatic Insulin Producing Tumor An insulin-producing neuroendocrine tumor arising from the beta cells of the pancreas. It may or may not be associated with inappropriate secretion of insulin and an associated clinical syndrome. Undetermined C5726 Pancreatic Intraductal Papillary-Mucinous Neoplasm with an Associated Invasive Carcinoma Pancreatic Intraductal Papillary Mucinous Neoplasm with an Associated Invasive Carcinoma A pancreatic intraductal papillary mucinous neoplasm characterized by the presence of a focal or multifocal invasive carcinomatous component. The invasive carcinoma is either colloid or ductal adenocarcinoma. Malignant C95507 Pancreatic Intraductal Tubulopapillary Neoplasm with an Associated Invasive Carcinoma A pancreatic intraductal tubulopapillary neoplasm characterized by the presence of an invasive carcinomatous component. The invasive carcinoma has a tubular pattern. Malignant C95582 Pancreatic Large Cell Neuroendocrine Carcinoma Pancreatic Large Cell NEC || Pancreatic Large Cell NEC G3 An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the pancreas. It is characterized by the presence of malignant large cells. Malignant C95471 Pancreatic Macrocystic Serous Cystadenoma A benign, non-metastasizing epithelial neoplasm arising from the exocrine pancreas. It is characterized by the presence of a few large cysts and is composed of glycogen-rich epithelial cells which produce a watery fluid. Benign C95458 Pancreatic Mixed Acinar-Ductal Carcinoma Mixed Acinar-Ductal Carcinoma of the Pancreas A carcinoma that arises from the pancreas showing either extensive mucin accumulation and acinar neoplastic cells or a mixture of columnar or signet-ring cells and acinar neoplastic cells. Malignant C3770 Pancreatic Neuroendocrine Carcinoma High Grade Pancreatic Neuroendocrine Carcinoma || High-Grade Pancreatic Neuroendocrine Carcinoma || Islet Cell Carcinoma || Pancreatic Endocrine Carcinoma || Pancreatic NEC || Pancreatic NEC G3 || Poorly Differentiated Neuroendocrine Neoplasm || Poorly Differentiated Pancreatic Endocrine Carcinoma An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the pancreas. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. Malignant C27720 Pancreatic Neuroendocrine Tumor Islet Cell Tumor || Pancreatic NET || Well Differentiated Pancreatic Endocrine Neoplasm || Well Differentiated Pancreatic Endocrine Tumor A low or intermediate grade well differentiated tumor with neuroendocrine differentiation that arises from the pancreas. According to the presence or absence of clinical syndromes that result from hormone hypersecretion, pancreatic neuroendocrine tumors are classified either as functional or nonfunctional. Undetermined C95584 Pancreatic Neuroendocrine Tumor G1 Pancreatic NET G1 || Serotonin-Producing Tumors With and Without Carcinoid Syndrome A low grade well differentiated tumor with neuroendocrine differentiation that arises from the pancreas. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal or less than 2%. Undetermined C45835 Pancreatic Neuroendocrine Tumor G2 Pancreatic NET G2 || Well Differentiated Pancreatic Endocrine Carcinoma || Well Differentiated Pancreatic Neuroendocrine Carcinoma An intermediate grade well differentiated tumor with neuroendocrine differentiation that arises from the pancreas. The mitotic count is 2-20 per 10 HPF and/or the Ki67 index is 3-20%. Undetermined C5712 Pancreatic Serous Cystadenoma Pancreatic Microcystic Adenoma || Serous Cystadenoma of Pancreas || Serous Cystadenoma of the Pancreas A benign, non-metastasizing cystic epithelial neoplasm arising from the exocrine pancreas. It is composed of glycogen-rich epithelial cells which produce a watery fluid. Signs and symptoms include abdominal mass, abdominal pain, nausea, vomiting, and weight loss. Benign C95583 Pancreatic Small Cell Neuroendocrine Carcinoma Pancreatic Small Cell Carcinoma || Pancreatic Small Cell NEC || Pancreatic Small Cell NEC G3 An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the pancreas. It is characterized by the presence of malignant small cells. Malignant C27454 Pancreatic VIP-Producing Neuroendocrine Tumor Pancreatic VIP Producing NET || Pancreatic VIP Producing Neoplasm || Pancreatic VIP Producing Tumor || Pancreatic Vasoactive Intestinal Peptide Producing Neoplasm || Pancreatic Vasoactive Intestinal Peptide Producing Tumor A usually malignant pancreatic neuroendocrine tumor producing vasoactive intestinal peptide (VIP). It may or may not be associated with inappropriate secretion of VIP and an associated clinical syndrome. Undetermined C2927 Papillary Carcinoma A malignant epithelial neoplasm characterized by a papillary growth pattern. A papillary carcinoma may be composed of glandular cells (papillary adenocarcinoma), squamous cells (papillary squamous cell carcinoma), or transitional cells (papillary transitional cell carcinoma). Bladder carcinoma is a representative example of papillary transitional cell carcinoma. Malignant C4319 Papillary Ependymoma A rare variant of ependymoma characterized by well formed papillae. Tumor cell processes abutting capillaries are usually GFAP-positive. Differential diagnoses include choroid plexus papilloma, papillary meningioma and metastatic papillary carcinoma. (Adapted from WHO) Undetermined C92554 Papillary Glioneuronal Tumor Papillary Glioneuronal Tumor (WHO Grade I) A WHO grade I, indolent and relatively circumscribed brain tumor. Morphologically it is characterized by the presence of astrocytes that line vascular and hyalinized pseudopapillae. In between the pseudopapillae aggregates of neurocytes, large neurons, and ganglioid cells are present. Undetermined C7526 Papillary Intralymphatic Angioendothelioma Dabska Tumor || Malignant Endothelial Papillary Angioendothelioma || Papillary Endovascular Angioendothelioma An intermediate, rarely metastasizing blood vessel neoplasm. It is characterized by the presence of lymphatic-like vascular channels and papillary endothelial proliferation. Undetermined C3904 Papillary Meningioma A WHO grade III meningioma characterized by the predominance of a perivascular pseudopapillary pattern. Malignant C6975 Papillary Renal Cell Carcinoma Also known as chromophil carcinoma, it represents a minority of renal cell carcinomas. It can be hereditary or sporadic. The sporadic papillary renal cell carcinoma is characterized by trisomy of chromosomes 7, 16, and 17, and loss of chromosome Y. The peak incidence is in the sixth and seven decades. It is classified as type 1 or 2, based on the cytoplasmic volume and the thickness of the lining neoplastic cells. The prognosis is more favorable than for conventional (clear cell) renal cell carcinoma. Malignant C92624 Papillary Tumor of the Pineal Region A rare tumor that arises from the pineal region and affects adults. It is characterized by the presence of neuroepithelial cells and a papillary architecture. Electron microscopic studies suggest ependymal differentiation. The clinical course is variable. Undetermined C7440 Papilloma A benign epithelial neoplasm that projects above the surrounding epithelial surface and consists of villous or arborescent outgrowths of fibrovascular stroma. Benign C3308 Paraganglioma Neoplasm of Paraganglion || Neoplasm of the Paraganglion || Paraganglion Neoplasm || Paraganglion Tumor || Paraganglionic Neoplasm || Paraganglionic Tumor || Tumor of Paraganglion || Tumor of the Paraganglion A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases. Undetermined C8193 Paranasal Sinus Squamous Cell Carcinoma Epidermoid Carcinoma of Paranasal Sinus || Epidermoid Carcinoma of the Paranasal Sinus || Paranasal Sinus Epidermoid Carcinoma || Squamous Cell Carcinoma of Paranasal Sinus || Squamous Cell Carcinoma of the Paranasal Sinus A squamous cell carcinoma that arises from the mucosal epithelial surface of the ethmoid, frontal, maxillary, or sphenoid sinus. Patients may present with nasal fullness, obstruction, and/or epistaxis. Malignant C3916 Parathyroid Gland Adenoma Adenoma of Parathyroid || Adenoma of the Parathyroid Gland || Parathyroid Adenoma A neoplasm arising from the parenchymal cells of the parathyroid glands. In the vast majority of cases, the tumor involves a single parathyroid gland. It is associated with the symptoms of primary hyperparathyroidism, resulting from the excessive production of parathyroid hormone. It is usually surrounded by a well-defined capsule. Capsular invasion, vascular invasion, and perineural invasion are absent. Overall cure rates are excellent following excision of the abnormal gland(s). Undetermined C4906 Parathyroid Gland Carcinoma Adenocarcinoma of Parathyroid || Adenocarcinoma of Parathyroid Gland || Adenocarcinoma of the Parathyroid || Adenocarcinoma of the Parathyroid Gland || Cancer of Parathyroid Gland || Cancer of the Parathyroid Gland || Carcinoma of Parathyroid || Carcinoma of Parathyroid Gland || Carcinoma of the Parathyroid || Carcinoma of the Parathyroid Gland || Parathyroid Adenocarcinoma || Parathyroid Cancer || Parathyroid Carcinoma || Parathyroid Gland Adenocarcinoma || Parathyroid Gland Cancer A malignant tumor arising from the parenchymal cells of the parathyroid gland. It is associated with the symptoms of primary hyperparathyroidism, resulting from the excessive production of parathyroid hormone. Morphologically, the differential diagnosis from parathyroid gland adenoma may be difficult. A definitive diagnosis of carcinoma is made only in the presence of capsular invasion, vascular invasion, and/or perineural invasion. Malignant C5941 Parotid Gland Adenocarcinoma Adenocarcinoma of Parotid || Adenocarcinoma of Parotid Gland || Adenocarcinoma of the Parotid || Adenocarcinoma of the Parotid Gland || Parotid Adenocarcinoma An adenocarcinoma that arises from the parotid gland. Representative examples include acinic cell carcinoma, basal cell adenocarcinoma, cystadenocarcinoma, and oncocytic carcinoma. Malignant C5937 Parotid Gland Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Parotid || Adenoid Cystic Carcinoma of Parotid Gland || Adenoid Cystic Carcinoma of the Parotid || Adenoid Cystic Carcinoma of the Parotid Gland || Parotid Adenoid Cystic Carcinoma An aggressive carcinoma that arises from the parotid gland. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Patients develop pain because of the tendency of these carcinomas to invade perineural tissues. Malignant C6791 Parotid Gland Carcinoma Carcinoma of Parotid || Carcinoma of Parotid Gland || Carcinoma of the Parotid || Carcinoma of the Parotid Gland || Parotid Carcinoma A carcinoma that arises from the parotid gland. Representative examples include mucoepidermoid carcinoma, adenoid cystic carcinoma, adenocarcinoma, and carcinoma ex pleomorphic adenoma. Malignant C5974 Parotid Gland Carcinoma ex Pleomorphic Adenoma Carcinoma ex Pleomorphic Adenoma of Parotid || Carcinoma ex Pleomorphic Adenoma of Parotid Gland || Carcinoma ex Pleomorphic Adenoma of the Parotid || Carcinoma ex Pleomorphic Adenoma of the Parotid Gland || Parotid Carcinoma ex Pleomorphic Adenoma A carcinoma that arises from a pleomorphic adenoma in the parotid gland. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive. Malignant C35832 Parotid Gland Carcinosarcoma An aggressive, high grade malignant tumor that arises from the parotid gland. It is characterized by the presence of a malignant epithelial and a sarcomatous component. Malignant C6847 Parotid Gland Monomorphic Adenoma Monomorphic Adenoma of Parotid || Monomorphic Adenoma of Parotid Gland || Monomorphic Adenoma of the Parotid || Monomorphic Adenoma of the Parotid Gland || Parotid Monomorphic Adenoma A benign epithelial neoplasm that arises from the parotid gland. It is characterized by the presence of a monomorphic cellular infiltrate. Benign C5938 Parotid Gland Mucoepidermoid Carcinoma Mucoepidermoid Carcinoma of Parotid || Mucoepidermoid Carcinoma of Parotid Gland || Mucoepidermoid Carcinoma of the Parotid || Mucoepidermoid Carcinoma of the Parotid Gland || Parotid Mucoepidermoid Carcinoma A carcinoma that arises from the parotid gland. It usually presents as a firm and painless mass. It is characterized by the presence of epidermoid cells, mucus producing cells, and cells of intermediate type. It usually spreads to pre-auricular lymph nodes. The majority of cases have a favorable outcome. Malignant C4650 Parotid Gland Pleomorphic Adenoma Benign Mixed Tumor of Parotid Gland || Benign Mixed Tumor of the Parotid Gland || Parotid Gland Benign Mixed Tumor || Parotid Pleomorphic Adenoma || Pleomorphic Adenoma of Parotid || Pleomorphic Adenoma of Parotid Gland || Pleomorphic Adenoma of the Parotid || Pleomorphic Adenoma of the Parotid Gland A benign, slow-growing tumor that arises from the parotid gland. It is composed of cells that demonstrate both epithelial and mesenchymal differentiation. It is the most common neoplasm of salivary gland origin and women are more often affected than men. Benign C5942 Parotid Gland Squamous Cell Carcinoma Parotid Squamous Cell Carcinoma || Squamous Cell Carcinoma of Parotid || Squamous Cell Carcinoma of Parotid Gland || Squamous Cell Carcinoma of the Parotid || Squamous Cell Carcinoma of the Parotid Gland An invasive squamous cell carcinoma that arises from the parotid gland. It usually affects elderly patients and presents as a rapidly enlarging tumor mass, often associated with pain. Malignant C5946 Parotid Gland Undifferentiated Carcinoma Anaplastic Carcinoma of Parotid || Anaplastic Carcinoma of Parotid Gland || Anaplastic Carcinoma of the Parotid || Anaplastic Carcinoma of the Parotid Gland || Anaplastic Parotid Carcinoma || Anaplastic Parotid Gland Carcinoma || Undifferentiated Carcinoma of Parotid || Undifferentiated Carcinoma of Parotid Gland || Undifferentiated Carcinoma of the Parotid || Undifferentiated Carcinoma of the Parotid Gland || Undifferentiated Parotid Gland Carcinoma A carcinoma that arises from the parotid gland and is characterized by the presence of undifferentiated, anaplastic malignant epithelial cells. Malignant C66865 Parotid Gland Warthin Tumor Parotid Gland Adenolymphoma || Parotid Gland Papillary Cystadenoma Lymphomatosum || Parotid Gland Warthin's Tumor An adenoma that arises from the parotid gland. It is characterized by an oncocytic, often papillary, epithelial component, dense lymphoid stroma, and cystic spaces. A strong association with smoking has been reported. It typically presents as a painless swelling in the lower portion of the parotid gland. Benign C80299 Pediatric Nodal Marginal Zone Lymphoma Childhood Nodal Marginal Zone Lymphoma A nodal marginal zone lymphoma affecting the pediatric population, predominantly males. It presents with asymptomatic and localized (mainly head and neck) disease in the vast majority of cases. Morphologically there is often progressive transformation of the germinal centers. The prognosis is excellent. Malignant C80297 Pediatric-Type Follicular Lymphoma A nodal follicular lymphoma with favorable prognosis. It lacks BCL-2 rearrangement and 14;18 translocation. Nearly all cases are localized. It is usually seen in the pediatric population but similar lymphomas may occur in adults. Malignant C39961 Penile Basal Cell Carcinoma Basal Cell Carcinoma of the Penis A basal cell carcinoma of the penis with an indolent clinical course. It is usually superficial and arises from the shaft and rarely the glans. Malignant C39963 Penile Bowenoid Papulosis A cutaneous, usually multifocal lesion in the penis characterized by the presence of verrucous papules. It is caused by human papillomavirus infection. Morphologically there is bowenoid dysplasia present. Progression to invasive carcinoma is infrequent. Undetermined C9061 Penile Carcinoma Cancer of Penis || Cancer of the Penis || Carcinoma of Penis || Carcinoma of the Penis || Penile Cancer A carcinoma that arises from the penis. Risk factors include phimosis and human papillomavirus infection. The majority of penile carcinomas are squamous cell carcinomas. The most frequent clinical presentation is an irregular mass in the glans of the penis. Treatment includes surgical management and radiation therapy. Malignant C27817 Penile Paget Disease Paget Disease of the Penis || Paget's Disease of Penis || Paget's Disease of the Penis A rare, slow-growing, usually non-invasive intraepithelial adenocarcinoma affecting the penile skin or mucosal surface. The malignant cells are large with abundant pale cytoplasm and vesicular nuclei with prominent nucleoli. (WHO 2016) Malignant C27322 Periampullary Adenocarcinoma An adenocarcinoma that arises from the periampullary region. Malignant C7631 Pericardial Malignant Mesothelioma Malignant Mesothelioma of Pericardium || Malignant Mesothelioma of the Pericardium A rare malignant mesothelioma that arises from the pericardium. Clinical presentation includes pericardial effusion, congestive heart failure, a mass or cardiac tamponade. Surgical resection is the treatment of choice. Adjunct treatments of chemotherapy or radiation have not improved outcomes. The prognosis is extremely poor due to its late presentation and difficulty obtaining complete surgical excision. In most cases the diagnosis is made at autopsy or postoperatively. Malignant C4973 Perineurioma A rare benign tumor composed entirely of neoplastic perineurial cells. It may occur in the soft tissues, intraneurally or in mucosal sites. Benign C4302 Periosteal Chondroma Juxtacortical Chondroma A benign neoplasm of bone surface composed of hyaline cartilage. It arises beneath the periosteum and is characterized by the presence of chondrocytes, a lobulated growth pattern, and calcification. Benign C7357 Periosteal Chondrosarcoma A chondrosarcoma arising from the surface of bone. It is characterized by a lobulated growth pattern, high mitotic activity, myxoid stroma formation, and necrotic changes. It occurs in adults. Clinical presentation includes pain, and sometimes swelling. Malignant C6594 Peripheral Ganglioneuroblastoma PNS Ganglioneuroblastoma || Peripheral Nervous System Ganglioneuroblastoma A ganglioneuroblastoma arising from the peripheral nervous system. Malignant C6591 Peripheral Neuroblastoma A neuroblastoma arising from the peripheral nervous system. Malignant C9341 Peripheral Primitive Neuroectodermal Tumor Peripheral Neuroectodermal Neoplasm || Peripheral Neuroectodermal Tumor || Peripheral Neuroepithelioma || Peripheral PNET || Peripheral Primitive Neuroectodermal Neoplasm A small round cell tumor with neural differentiation arising from the soft tissues or bone. Malignant C8776 Peripheral Primitive Neuroectodermal Tumor of Bone Bone Peripheral Neuroepithelioma || Osseous Peripheral Neuroepithelioma || Peripheral Neuroectodermal Tumor of Bone || Peripheral Neuroectodermal Tumor of the Bone || Peripheral Neuroepithelioma of Bone || Peripheral Neuroepithelioma of the Bone A small round cell tumor with neural differentiation arising from the bone. It may be associated with pain. Malignant C27471 Peripheral Primitive Neuroectodermal Tumor of Soft Tissues Peripheral Neuroectodermal Tumor of Soft Tissues || Peripheral Neuroectodermal Tumor of the Soft Tissues || Peripheral Neuroepithelioma of Soft Tissues || Peripheral Neuroepithelioma of the Soft Tissues A small round cell tumor with neural differentiation arising from the soft tissues. Malignant C4340 Peripheral T-Cell Lymphoma, Not Otherwise Specified Peripheral T-Cell Lymphoma, NOS A heterogenous category of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the specifically defined entities of mature T-cell lymphoma in the 2017 WHO classification. Excluded from this category are tumors with a T follicular helper (TFH) cell phenotype. Variants include lymphoepithelioid lymphoma (Lennert lymphoma) and primary EBV-positive nodal T-cell or NK-cell lymphoma. The follicular variant included in the peripheral T-cell lymphomas, not otherwise specified, in the 2008 edition of the WHO classification has been moved to the category of angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin in the 2017 WHO update. The same is true for a proportion of cases previously designated as the T-zone variant, because they usually have a TFH-cell phenotype. (WHO 2017) Malignant C9350 Peritoneal Malignant Mesothelioma Malignant Mesothelioma of Peritoneum || Malignant Mesothelioma of the Peritoneum || Malignant Peritoneal Mesothelioma An aggressive malignant mesothelioma that arises from the peritoneum. Patients usually present with abdominal pain and ascites. Malignant C6536 Peritoneal Multicystic Mesothelioma Multicystic Mesothelioma of Peritoneum || Multicystic Mesothelioma of the Peritoneum || Multilocular Peritoneal Inclusion Cyst A cystic mesothelioma that arises from the peritoneum and usually affects young to middle aged females. Grossly, it presents as a large multiloculated tumor mass, usually in the pelvic peritoneum. Histologically it is characterized by the presence of multiple cysts that are lined by one or more layers of mesothelial cells that do not show atypia. Patients usually present with abdominal or pelvic mass and pain. The clinical course is indolent. The tumor may recur, but transformation to diffuse malignant mesothelioma is rare. Uncertain Malignant Potential C45661 Peritoneal Well Differentiated Papillary Mesothelial Tumor Peritoneal WDPM || Peritoneal WDPMT || Peritoneal Well Differentiated Papillary Mesothelioma || Peritoneal Well-Differentiated Papillary Mesothelial Tumor || Peritoneal Well-Differentiated Papillary Mesothelioma A localized or multifocal mesothelioma arising from the peritoneum. It predominantly occurs in women. It is characterized by the formation of papillae, covered by a single layer of blunt mesothelial cells. Mitotic figures are not present. There is no evidence of severe cytologic atypia. It has a relatively favorable clinical outcome, compared to diffuse malignant mesothelioma. Uncertain Malignant Potential C102872 Pharyngeal Squamous Cell Carcinoma A squamous cell carcinoma that arises from the pharynx. Malignant C9129 Philadelphia Chromosome Negative, BCR-ABL1 Positive Chronic Myelogenous Leukemia Ph Negative Chronic Myelogenous Leukemia || Ph' Chromosome Negative Chronic Granulocytic Leukemia || Ph' Chromosome Negative Chronic Myelocytic Leukemia || Ph' Chromosome Negative Chronic Myeloid Leukemia || Ph' Negative Chronic Granulocytic Leukemia || Ph- Chronic Myelogenous Leukemia || Ph1 Chromosome Negative Chronic Myelocytic Leukemia || Ph1 Chromosome Negative Chronic Myelogenous Leukemia || Philadelphia Chromosome Negative BCR-ABL1 Positive Chronic Myelogenous Leukemia || Philadelphia Chromosome Negative Chronic Granulocytic Leukemia || Philadelphia Chromosome Negative Chronic Myelocytic Leukemia || Philadelphia Chromosome Negative Chronic Myelogenous Leukemia || Philadelphia Chromosome Negative Chronic Myeloid Leukemia A chronic myelogenous leukemia which does not have the characteristic t(9;22)(q34;q11.2) translocation but it has either a variant translocation or a cryptic translocation that can not be detected by conventional cytogenetic analysis. In such cases the BCR-ABL1 fusion gene is always detected by RT-PCR, FISH, or Southern blot analysis. Malignant C9128 Philadelphia Chromosome Positive, BCR-ABL1 Positive Chronic Myelogenous Leukemia Chronic Myeloid Leukemia t(9;22) (q34;q11), BCR/ABL Positive || Ph' Chromosome Positive Chronic Myelocytic Leukemia || Ph' Chromosome Positive Chronic Myelogenous Leukemia || Ph' Chromosome Positive Chronic Myeloid Leukemia || Ph' Positive Chronic Granulocytic Leukemia || Ph1 Chromosome Positive Chronic Myelocytic Leukemia || Ph1 Chromosome Positive Chronic Myelogenous Leukemia || Ph1 Chromosome Positive Chronic Myeloid Leukemia || Ph1 Positive Chronic Granulocytic Leukemia || Philadelphia Chromosome Positive CML || Philadelphia Chromosome Positive Chronic Granulocytic Leukemia || Philadelphia Chromosome Positive Chronic Myelocytic Leukemia || Philadelphia Chromosome Positive Chronic Myelogenous Leukemia || Philadelphia Chromosome Positive Chronic Myeloid Leukemia A chronic myelogenous leukemia characterised by the t(9;22)(q34;q11) chromosomal translocation, resulting in the presence of the Philadelphia chromosome and the BCR-ABL1 fusion gene. Malignant C121974 Ph-Like Acute Lymphoblastic Leukemia Ph-Like ALL A leukemia subtype characterized by a gene-expression profile similar to that of BCR-ABL1-positive acute lymphoblastic leukemia, alterations of lymphoid transcription factor genes, and a poor outcome. Malignant C2977 Phyllodes Tumor Cystosarcoma Phyllodes || Phyllodes Neoplasm A benign, borderline, or malignant fibroepithelial neoplasm arising from the breast and rarely the prostate gland. It may recur following resection. The recurrence rates are higher for borderline and malignant phyllodes tumors. In borderline and malignant phyllodes tumors metastases to distant anatomic sites can occur. The incidence of metastases is higher in malignant phyllodes tumors. Undetermined C4047 Pilocytic Astrocytoma Grade I Astrocytic Neoplasm || Grade I Astrocytic Tumor || Grade I Astrocytoma A WHO grade I, relatively circumscribed, slowly growing, often cystic astrocytoma occurring in children and young adults. Histologically it is characterized by a biphasic pattern with compacted bipolar cells associated with Rosenthal fibers and multipolar cells associated with microcysts and eosinophilic bodies/hyaline droplets. (WHO) Undetermined C4114 Pilomatrical Carcinoma Invasive Pilomatrixoma || Matrical Carcinoma || Pilomatrix Carcinoma || Pilomatrix Carcinoma of Skin || Pilomatrix Carcinoma of the Skin || Pilomatrix Skin Carcinoma A very rare, locally aggressive, malignant neoplasm of the hair follicle. The majority of the cases arise de novo, however malignant transformation from a pre-existing pilomatricoma has been reported. It usually presents as a solitary nodule in the head and neck, upper extremities, or buttocks. Morphologically, it is characterized by the presence of aggregates of basaloid cells infiltrating the dermis. Masses of ghost cells are present in the cellular aggregates. Complete surgical excision is the treatment of choice. If it is not completely removed, it usually recurs, but it rarely metastasizes to distant anatomic sites. Malignant C7368 Pilomatricoma Benign Pilomatricoma || Benign Pilomatrixoma || Calcifying Epithelioma of Malherbe || Pilomatrixoma A benign adnexal neoplasm arising from hair-bearing skin surfaces, usually the head and neck and upper extremities. It usually presents as a solitary, slow-growing nodular mass. Morphologically, it displays differentiation towards the matrix and inner sheath of the normal hair follicle and the hair cortex. Complete surgical excision is usually curative. Occasionally, it may recur. Benign C40315 Pilomyxoid Astrocytoma An astrocytic tumor of uncertain relation to pilocytic astrocytoma. It occurs predominantly in infants and young children. It is characterized by a monomorphic architectural pattern, usually associated with the absence of Rosenthal fibers and eosinophilic granular bodies. The clinical course is usually aggressive. Undetermined C6965 Pineal Parenchymal Cell Neoplasm Neoplasm of Pineal Gland || Neoplasm of the Pineal Gland || Pineal Gland Neoplasm || Pineal Gland Tumor || Pineal Parenchymal Cell Tumor || Pineal Parenchymal Neoplasm || Pineal Parenchymal Tumor || Pineocytic Neoplasm || Pineocytic Tumor || Tumor of Pineal Gland || Tumor of the Pineal Gland A neoplasm arising from the pineocyte, a cell with photosensory and neuroendocrine functions. It may be composed of mature elements or primitive, immature cells. The cellular composition determines the biological behavior and clinical outcome. Three types are recognized: pineoblastoma, pineocytoma, and pineal parenchymal tumor of intermediate differentiation (Adapted from WHO.) Undetermined C6967 Pineal Parenchymal Tumor of Intermediate Differentiation A WHO grade II or III pineal parenchymal neoplasm of intermediate-grade malignancy, affecting all ages. It is composed of diffuse sheets or large lobules of uniform cells with mild to moderate nuclear atypia and low to moderate level mitotic activity. (Adapted from WHO) Undetermined C9344 Pineoblastoma PNET of Pineal Gland || PNET of the Pineal Gland || Pineal Gland PNET || Pineal Gland Primitive Neuroectodermal Neoplasm || Pineal Gland Primitive Neuroectodermal Tumor || Pineal PNET || Pineal Primitive Neuroectodermal Neoplasm || Pineal Primitive Neuroectodermal Tumor || Pineoblastoma (WHO Grade IV) || Primitive Neuroectodermal Neoplasm of Pineal Gland || Primitive Neuroectodermal Neoplasm of the Pineal Gland || Primitive Neuroectodermal Tumor of Pineal Gland || Primitive Neuroectodermal Tumor of the Pineal Gland A poorly differentiated malignant embryonal neoplasm arising from the pineal region. It usually occurs in children and it is characterized by the presence of small immature neuroepithelial cells. It may follow an aggressive clinical course. Malignant C6966 Pineocytoma Pineocytoma (WHO Grade I) A WHO grade I slow growing tumor, more frequently affecting young adults. It is composed of small, uniform, mature cells resembling pineocytes with occasional large pineocytomatous rosettes. It may show a wide range of divergent phenotypes, including neuronal, glial, melanocytic, photoreceptor and mesenchymal differentiation. Pineocytoma generally has a relatively favorable prognosis. (Adapted from WHO) Benign C3329 Pituitary Gland Adenoma Adenoma of Pituitary || Adenoma of Pituitary Gland || Adenoma of the Pituitary || Adenoma of the Pituitary Gland || Pituitary Adenoma A non-metastasizing tumor that arises from the adenohypophysial cells of the anterior lobe of the pituitary gland. The tumor can be hormonally functioning or not. The diagnosis can be based on imaging studies and/or radioimmunoassays. Due to its location in the sella turcica, expansion of the tumor mass can impinge on the optic chiasm or involve the temporal lobe, third ventricle and posterior fossa A frequently associated physical finding is bitemporal hemianopsia which may progress to further visual loss. Undetermined C4536 Pituitary Gland Carcinoma Cancer of Pituitary || Cancer of Pituitary Gland || Cancer of the Pituitary || Cancer of the Pituitary Gland || Carcinoma of Pituitary || Carcinoma of Pituitary Gland || Carcinoma of the Pituitary || Carcinoma of the Pituitary Gland || Pituitary Carcinoma || Pituitary Gland Adenocarcinoma || Pituitary Gland Cancer A rare adenocarcinoma with poor prognosis, arising from the adenohypophysial cells of the anterior lobe of the pituitary gland or pre-existing adenomas. The majority are hormonally functioning neoplasms, usually producing prolactin or ACTH. The diagnosis is based on the presence of metastases. Syndromes associated with pituitary gland carcinomas include hyperprolactinemia, Cushing disease, and acromegaly. Malignant C43542 Pituitary Gland Macroadenoma A pituitary gland adenoma with a diameter greater than 10 mm. Clinical manifestations include headache, visual field disturbances, pituitary insufficiency, and mild hyperprolactinemia. Benign C43541 Pituitary Gland Microadenoma A pituitary gland adenoma with a diameter equal or less than 10 mm. Benign C3180 Plasma Cell Leukemia Leukemia Plasmacytic || Plasmacytic Leukemia An aggressive plasma cell neoplasm characterized by the presence of neoplastic plasma cells in the peripheral blood. It is characterized by the presence of a circulating clonal plasma cell count that exceeds 2x10^9/L or is 20% of the leukocyte differential count. Malignant C3242 Plasma Cell Myeloma Multiple Myeloma || Myeloma A bone marrow-based plasma cell neoplasm characterized by a serum monoclonal protein and skeletal destruction with osteolytic lesions, pathological fractures, bone pain, hypercalcemia, and anemia. Clinical variants include non-secretory myeloma, smoldering myeloma, indolent myeloma, and plasma cell leukemia. (WHO, 2001) Malignant C7242 Plasma Cell Myeloma Post-Transplant Lymphoproliferative Disorder Plasma Cell Myeloma PTLD A lymphoproliferative disorder that develops following an organ transplantation and exhibits plasma cell myeloma features. Malignant C7224 Plasmablastic Lymphoma An aggressive diffuse large B-cell lymphoma frequently arising in the setting of HIV infection and characterized by the presence of large neoplastic cells resembling B-immunoblasts which have the immunophenotypic profile of plasma cells. Sites of involvement include the oral cavity, sinonasal cavity, skin, soft tissues, gastrointestinal tract, and bone. Malignant C9349 Plasmacytoma A malignant (clonal) proliferation of plasma cells that are cytologically and immunophenotypically identical to those of plasma cell myeloma, but manifest a localized osseous or extraosseous growth pattern. (WHO, 2001) Malignant C8602 Pleomorphic Adenoma A neoplasm characterized by the presence of benign epithelial and myoepithelial cells and a mesenchymal component that may contain mucoid, myxoid, cartilaginous, or osseous areas. It may be completely or partially encapsulated. It occurs in the parotid gland, submandibular gland, minor salivary glands in the oral cavity, upper respiratory tract, and nasal cavity and paranasal sinuses. It usually presents as a slow growing painless mass. Infrequently, patients may present with pain and facial palsy. It may recur after excision or transform to a malignant neoplasm (carcinoma ex pleomorphic adenoma). Benign C3705 Pleomorphic Liposarcoma A rare, aggressive liposarcoma characterized by the presence of varying proportions of pleomorphic lipoblasts in a background that resembles undifferentiated pleomorphic sarcoma. Malignant C4258 Pleomorphic Rhabdomyosarcoma An aggressive malignant mesenchymal neoplasm with skeletal muscle differentiation, occurring in adults and rarely in children. The tumor is characterized by the presence of bizarre round, spindle, and polygonal cells. Clinical presentation includes a rapidly enlarging painful mass usually of the lower extremities. Malignant C39747 Pleomorphic Variant Mantle Cell Lymphoma An aggressive mantle cell lymphoma characterized by the presence of pleomorphic neoplastic B-lymphocytes. Malignant C4323 Pleomorphic Xanthoastrocytoma Pleomorphic Xantho-Astrocytoma A WHO grade ll astrocytic tumor with a relatively favorable prognosis. It is characterized by pleomorphic and lipidized cells expressing GFAP often surrounded by a reticulin network and eosinophilic granular bodies. It presents in the superficial cerebral hemispheres and involves the meninges. It typically affects children and young adults. Undetermined C7376 Pleural Malignant Mesothelioma Malignant Mesothelioma of Pleura || Malignant Mesothelioma of the Pleura || Malignant Pleural Mesothelioma || Pleural Diffuse Malignant Mesothelioma A malignant neoplasm that arises from mesothelial cells in the pleura and shows a diffuse growth pattern. It arises on the parietal and sometimes visceral pleura as multiple small nodules that later become confluent and invade the chest wall adipose tissue and muscle. Asbestos exposure is the main cause for the development of pleural malignant mesothelioma. It usually affects patients over sixty years of age. The latency period is long. Patients usually present with pleural effusion, dyspnea and chest wall pain. Additional signs and symptoms include chills, sweating, weight loss, and weakness. Morphologic variants include epithelioid, desmoplastic, sarcomatoid, and biphasic mesothelioma. The clinical course is usually aggressive. Malignant C45660 Pleural Well Differentiated Papillary Mesothelial Tumor Pleural WDPM || Pleural WDPMT || Pleural Well Differentiated Papillary Mesothelioma || Pleural Well-Differentiated Papillary Mesothelial Tumor || Pleural Well-Differentiated Papillary Mesothelioma A rare, non-invasive, localized or multifocal neoplasm that arises from the pleura. It is characterized by the presence of papillae with myxoid fibrovascular cores, lined by a single layer of mesothelial cells. The clinical course is usually indolent. Uncertain Malignant Potential C5669 Pleuropulmonary Blastoma Pulmonary Blastoma of Childhood A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients with pleuropulmonary blastoma, there are other lesions or neoplasms that may affect patients or their families, including lung or kidney cysts, and ovarian or testicular neoplasms. Heterozygous germline mutations in DICER1 gene have been identified in families harboring pleuropulmonary blastomas. Malignant C45916 Plurihormonal Pituitary Gland Adenoma Multihormonal Adenoma || Multihormonal Pituitary Gland Adenoma || Plurihormonal Adenoma || Polyhormonal Adenoma An infrequent chromophobic or slightly eosinophilic pituitary gland adenoma that shows immunoreactivity for more than one pituitary hormone, excluding the following two combinations: growth hormone/prolactin/TSH, and FSH/LH. Benign C3336 Polycythemia Vera Polycythemia Rubra Vera A chronic myeloproliferative neoplasm characterized by an increased red blood cell production. The bone marrow is hypercellular due to a panmyelotic proliferation typically characterized by pleomorphic megakaryocytes. The major symptoms are related to hypertension, splenomegaly or to episodes of thrombosis and/or hemorrhage. Malignant C41232 Polycythemia Vera, Polycythemic Phase Polycythemia Vera, Polycythemic Stage Polycythemia vera characterized by the proliferation of the erythroid, granulocytic, and megakaryocytic lineages. The peripheral blood shows excess of red blood cells, neutrophilia, and thrombocytosis. The bone marrow is hypercellular for the patient's age. Malignant C41233 Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase Polycythemia Vera, "Spent" Phase || Polycythemia Vera, Post-Polycythemic Myelofibrosis and Myeloid Metaplasia Stage || Post-PV MF || Post-polycythemia Vera Myelofibrosis || Spent Phase and Post-Polycythemic Myelofibrosis Progression of polycythemia vera characterized by decreased erythropoiesis and granulopoiesis, reticulin and collagen fibrosis of the bone marrow, leucoerythroblastic reaction in the peripheral blood, poikilocytosis, tear-drop shaped red blood cells, and splenomegaly. Malignant C7183 Polymorphic Post-Transplant Lymphoproliferative Disorder Polymorphic B-Cell Lymphoma || Polymorphic PTLD A clonal B-cell lymphoproliferative disorder arising as a result of post-transplant immunosuppression therapy. It is characterized by destructive infiltration of lymph nodes and extranodal sites by a polymorphic B-cell infiltrate composed of small and medium sized lymphocytes, immunoblasts, and plasma cells. In some cases, reduction of the immunosuppression therapy may lead to regression of the lesions. In other cases the lesions may progress to lymphoma. Uncertain Malignant Potential C42047 Poorly Differentiated Neuroblastoma A neuroblastoma in which the differentiating neuroblasts constitute less than five-percent of the tumor cells. Malignant C27916 Poorly Differentiated Prostate Adenocarcinoma An invasive prostate adenocarcinoma characterized by the presence of poorly differentiated malignant cells. Malignant C6040 Poorly Differentiated Thyroid Gland Carcinoma Insular Carcinoma || Poorly Differentiated Carcinoma of Thyroid Gland || Poorly Differentiated Carcinoma of the Thyroid Gland || Poorly Differentiated Thyroid Carcinoma || Thyroid Gland Poorly Differentiated Carcinoma An adenocarcinoma arising from the thyroid gland showing only limited evidence of follicular cell differentiation. Microscopically, the adenocarcinoma cells are arranged in insular, solid, and trabecular patterns. There is associated necrosis, and vascular invasion. The prognosis depends on the tumor stage, complete or partial surgical removal of the tumor, and the degree of response to radioactive iodine therapy. (adapted from WHO Tumors of Endocrine Organs, IARC Press, Lyon 2004) Malignant C27273 Poroma Eccrine Poroma A benign, well circumscribed sweat gland neoplasm with eccrine or apocrine differentiation. It usually presents as a solitary, dome-shaped papule, nodule, or plaque on acral sites. It is characterized by a proliferation of uniform basaloid cells in the dermis and it is associated with the presence of focal ductal and cystic structures. Complete excision is curative. Benign C35693 Posterior Pharyngeal Wall Squamous Cell Carcinoma A squamous cell carcinoma that arises from the posterior wall of the pharynx. Malignant C8587 Precancerous Polyp A polyp with severe dysplastic features. Undetermined C41237 Prefibrotic/Early Primary Myelofibrosis Chronic Idiopathic Myelofibrosis, Cellular Phase || Chronic Idiopathic Myelofibrosis, Prefibrotic Stage || Primary Myelofibrosis, Prefibrotic Stage || Primary Myelofibrosis, Prefibrotic/Early Stage Primary myelofibrosis characterized by bone marrow hypercellularity and the presence of atypical megakaryocytes. There is no increase in the percentage of myeloblasts and no significant increase in reticulin or collagen fibrosis in the bone marrow. Malignant C4834 Primary Bone Osteosarcoma Primary Osteosarcoma of Bone A usually aggressive malignant bone-forming mesenchymal neoplasm arising from the bone. It usually involves the long bones and predominantly affects adolescents and young adults. Pain and a palpable mass are the most frequent clinical sign and symptom. It may spread to other anatomic sites, particularly the lungs. Malignant C7155 Primary Central Chondrosarcoma Primary Bone Chondrosarcoma || Primary Chondrosarcoma || Primary Chondrosarcoma of Bone || Primary Chondrosarcoma of the Bone A chondrosarcoma arising from the central portion of bone without a benign precursor. Malignant C6860 Primary Cutaneous Anaplastic Large Cell Lymphoma Primary Anaplastic Large Cell Lymphoma of Skin || Primary Anaplastic Large Cell Lymphoma of the Skin || Primary Cutaneous CD30 Positive Anaplastic Large Cell Lymphoma || Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma An anaplastic large cell lymphoma limited to the skin at the time of diagnosis. Most patients present with solitary or localized skin lesions, which may be tumors, nodules or papules. The t(2;5) translocation that is present in many cases of systemic anaplastic large cell lymphoma, is not found in this disease. (WHO, 2001) Malignant C6858 Primary Cutaneous B-Cell Non-Hodgkin Lymphoma Primary B-Cell Non-Hodgkin Lymphoma of Skin || Primary Cutaneous B-Cell Non-Hodgkin's Lymphoma A B-cell non-Hodgkin lymphoma arising from the skin. Representative examples include cutaneous marginal zone B-cell lymphoma and cutaneous follicle center lymphoma. Malignant C45366 Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder Primary Cutaneous CD4-Positive Small/Medium-Sized T-Cell Lymphoproliferative Disorder || Primary Cutaneous CD4-Positive Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder A primary cutaneous T-cell lymphoproliferative disorder. It usually presents with a solitary plaque or tumor on the face, neck, or upper trunk. Morphologically, it is composed of small to medium-sized CD4-positive, CD8-negative, and CD30-negative pleomorphic T-lymphocytes. A small number of large pleomorphic T-lymphocytes may also be present. The lymphocytic infiltrate is dermal. Focal epidermotropism and subcutaneous involvement may be present. The clinical behavior is almost always indolent and most patients present with localized disease. Uncertain Malignant Potential C45339 Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma Primary Cutaneous Aggressive Epidermotropic CD8-Positive T-Cell Lymphoma || Primary Cutaneous CD8-Positive Aggressive Epidermotropic T-Cell Lymphoma An aggressive, epidermotropic primary cutaneous T-cell lymphoma. It is characterized by the presence of eruptive papules and nodules, or tumors, which may be localized or disseminated. Morphologically, it is composed of small to medium-sized, or medium-sized to large, CD8-positive T-lymphocytes with pleomorphic or blastic nuclei. Epidermotropism is often pronounced. Angiocentricity and angioinvasion may be present. Malignant C45193 Primary Cutaneous Diffuse Large B-Cell Lymphoma A diffuse large B-cell lymphoma that arises from the skin. This category includes primary cutaneous diffuse large B-cell lymphoma, leg type, and primary cutaneous diffuse large B-cell lymphoma, other, which includes primary cutaneous intravascular large B-cell lymphoma and primary cutaneous T-cell/histiocyte-rich large B-cell lymphoma. Malignant C45194 Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type An aggressive primary cutaneous B-cell lymphoma, usually involving the lower leg. It is composed of a generally monotonous proliferation of immunoblasts, or less frequently centroblasts, with few admixed reactive cells. This type of lymphoma occurs most often in elderly women who present with rapidly growing tumors, usually on one or both legs. Dissemination to extracutaneous sites is frequent. Treatment with combination chemotherapy is usually required. Malignant C45213 Primary Cutaneous Diffuse Large B-Cell Lymphoma, Other A rare type of primary cutaneous diffuse large B-cell lymphoma that does not belong in the group of primary cutaneous diffuse large B-cell lymphomas, leg type, or the group of primary cutaneous follicle center lymphomas. It includes subtypes/entities of diffuse large B-cell lymphoma (e.g. intravascular large B-cell lymphoma, plasmablastic lymphoma, T-cell/histiocyte rich lymphoma) that can involve the skin as the primary anatomic site. Such cases, unlike their nodal counterparts, usually have an excellent prognosis. Malignant C7217 Primary Cutaneous Follicle Center Lymphoma Crosti's Disease || Cutaneous Follicle Center Lymphoma || Cutaneous Follicle Centre Lymphoma || Reticulohistiocytoma of the Dorsum A primary lymphoma of the skin composed of various numbers of small and large irregular neoplastic follicle center cells. Its morphologic pattern can be nodular, diffuse, or nodular and diffuse. It presents with solitary or grouped plaques and tumors, and it usually involves the scalp, forehead, or trunk. It rarely involves the legs. This type of cutaneous lymphoma tends to remain localized to the skin. Malignant C45340 Primary Cutaneous Gamma-Delta T-Cell Lymphoma Cutaneous Gamma/Delta T-Cell Lymphoma || Peripheral Gamma-Delta T-Cell Lymphoma An aggressive cutaneous lymphoma of mature, activated gamma/delta T-lymphocytes. It usually presents with disseminated plaques and nodules. Involvement of mucosal sites is frequent. However, involvement of lymph nodes, spleen, or bone marrow is uncommon. Morphologically, there are three patterns of cutaneous involvement: epidermotropic, dermal, and subcutaneous. Often, more than one pattern may co-exist in a single biopsy specimen, or may be present in different biopsy specimens from the same patient. The lymphocytic infiltrate is composed of medium- to large-sized lymphocytes expressing CD56. Most cases lack both CD4 and CD8, although CD8 may be present in some cases. This group of lymphomas includes cases previously known as subcutaneous panniculitis-like T-cell lymphoma with a gamma/delta phenotype. Malignant C7230 Primary Cutaneous Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Marginal Zone B Cell Lymphoma of Skin || Marginal Zone B Cell Lymphoma of the Skin || Primary Cutaneous Marginal Zone B Cell Lymphoma of Mucosa-Associated Lymphoid Tissue || Primary Cutaneous Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue || SALT Lymphoma || Skin-Associated Lymphoid Tissue Lymphoma A low-grade, extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue that arises from the skin. It usually presents with multifocal papular or nodular lesions in the arms or trunk. It rarely disseminates to internal organs or progresses to high grade lymphoma. Malignant C45332 Primary Cutaneous Peripheral T-Cell Lymphoma, Rare Subtype A rare, mature T-Cell lymphoma arising from the skin. Malignant C3467 Primary Cutaneous T-Cell Non-Hodgkin Lymphoma Cutaneous T Cell Lymphoma || Cutaneous T-Cell Non-Hodgkin Lymphoma || Cutaneous T-Cell Non-Hodgkin's Lymphoma || Primary Cutaneous T-Cell Non-Hodgkin's Lymphoma || Skin T-Cell Non-Hodgkin's Lymphoma || T-Cell Non-Hodgkin's Lymphoma of Skin || T-Cell Non-Hodgkin's Lymphoma of the Skin A T-cell non-Hodgkin lymphoma arising from the skin. Representative examples include mycosis fungoides and primary cutaneous anaplastic large cell lymphoma. Malignant C6915 Primary Effusion Lymphoma A large B-cell lymphoma usually presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV8), also called Kaposi sarcoma-associated herpesvirus. It mostly occurs in the setting of immunodeficiency. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. Rare HHV8-positive lymphomas indistinguishable from primary effusion lymphomas (PEL) present as solid tumor masses, and have been termed extracavitary PEL. The prognosis is extremely unfavorable. (WHO 2017) Malignant C9280 Primary Mediastinal (Thymic) Large B-Cell Lymphoma B-Cell Diffuse Large Cell Lymphoma of Mediastinum || B-Cell Diffuse Large Cell Lymphoma of the Mediastinum || Mediastinal B-Cell Diffuse Large Cell Lymphoma || Primary Mediastinal Large B-Cell Lymphoma A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis. Response to intensive chemotherapy, with or without radiotherapy, is usually good. (WHO, 2001) Malignant C2862 Primary Myelofibrosis Agnogenic Myeloid Metaplasia || Chronic Idiopathic Myelofibrosis || Idiopathic Bone Marrow Fibrosis || Idiopathic Myelofibrosis || Myelosclerosis with Myeloid Metaplasia A chronic myeloproliferative neoplasm characterized by bone marrow fibrosis, proliferation of atypical megakaryocytes and granulocytes in the bone marrow, anemia, splenomegaly, and extramedullary hematopoiesis. (WHO, 2001) Malignant C40022 Primary Peritoneal Carcinoma A rare carcinoma that arises from the peritoneum and resembles the malignant surface epithelial-stromal tumors that arise from the ovary. Serous adenocarcinoma is the most common histologic variant. It affects women almost exclusively. The diagnosis of primary peritoneal carcinoma can be made only if both ovaries are not involved by tumor, or, if the ovaries are involved, the tumor is confined to the ovarian surface without invasion of the ovarian stroma and the peritoneal involvement is greater than the ovarian surface involvement. Malignant C40023 Primary Peritoneal Serous Adenocarcinoma A rare, serous adenocarcinoma that arises from the lining of the peritoneum. It affects females. The clinical behavior and pathologic characteristics are similar to the serous adenocarcinoma that arises from the ovary. Malignant C7695 Primary Peritoneal Serous Papillary Adenocarcinoma Primary Peritoneal Serous Papillary Carcinoma || Primary Serous Papillary Carcinoma of Peritoneum A rare, serous papillary adenocarcinoma that arises from the lining of the peritoneum. It affects females. The clinical behavior and pathologic characteristics are similar to the serous papillary adenocarcinoma that arises from the ovary. Malignant C39898 Primary Prostate Urothelial Carcinoma Prostate Transitional Cell Carcinoma || Prostate Urothelial Carcinoma || Transitional Cell Carcinoma of Prostate || Transitional Cell Carcinoma of the Prostate An urothelial carcinoma that arises from the urothelial lining of the prostatic ducts or the prostatic urethra. Malignant C5962 Prolactin-Producing Pituitary Gland Carcinoma Malignant Pituitary Gland Prolactinoma || Malignant Pituitary Prolactinoma || Malignant Prolactin Producing Neoplasm of Pituitary || Malignant Prolactin Producing Neoplasm of Pituitary Gland || Malignant Prolactin Producing Neoplasm of the Pituitary || Malignant Prolactin Producing Neoplasm of the Pituitary Gland || Malignant Prolactin Producing Pituitary Gland Neoplasm || Malignant Prolactin Producing Pituitary Gland Tumor || Malignant Prolactin Producing Pituitary Neoplasm || Malignant Prolactin Producing Pituitary Tumor || Malignant Prolactin Producing Tumor || Malignant Prolactin Producing Tumor of Pituitary || Malignant Prolactin Producing Tumor of Pituitary Gland || Malignant Prolactin Producing Tumor of the Pituitary || Malignant Prolactin Producing Tumor of the Pituitary Gland || Malignant Prolactin Secreting Neoplasm of Pituitary || Malignant Prolactin Secreting Neoplasm of Pituitary Gland || Malignant Prolactin Secreting Neoplasm of the Pituitary || Malignant Prolactin Secreting Neoplasm of the Pituitary Gland || Malignant Prolactin Secreting Pituitary Gland Neoplasm || Malignant Prolactin Secreting Pituitary Gland Tumor || Malignant Prolactin Secreting Pituitary Neoplasm || Malignant Prolactin Secreting Pituitary Tumor || Malignant Prolactin Secreting Tumor of Pituitary || Malignant Prolactin Secreting Tumor of Pituitary Gland || Malignant Prolactin Secreting Tumor of the Pituitary || Malignant Prolactin Secreting Tumor of the Pituitary Gland || Malignant Prolactinoma || Malignant Prolactinoma of Pituitary || Malignant Prolactinoma of Pituitary Gland || Malignant Prolactinoma of the Pituitary || Malignant Prolactinoma of the Pituitary Gland || PRL Producing Pituitary Gland Carcinoma || Prolactin Producing Pituitary Gland Carcinoma A rare, hormonally functioning or non-functioning pituitary gland adenocarcinoma that produces prolactin. Malignant C3181 Prolymphocytic Leukemia A mature B- or T- cell leukemia with progressive clinical course. It is characterized by the presence of medium-sized lymphocytes with visible nucleoli (prolymphocytes) in the peripheral blood, bone marrow, and spleen. Malignant C111692 Proneural Glioblastoma A molecular subtype of glioblastoma that is associated with younger age at presentation and is characterized by p53 mutations and PDGFRa amplifications. Malignant C5596 Prostate Acinar Adenocarcinoma Acinar Adenocarcinoma of Prostate || Acinar Adenocarcinoma of the Prostate || Acinar Prostate Adenocarcinoma || Prostatic Acinar Adenocarcinoma An invasive adenocarcinoma of the prostate gland composed of secretory cells. It is the most common histologic type of prostate adenocarcinoma. Several morphologic variants exist, including atrophic, pseudohyperplastic, foamy gland, and oncocytic variants. Malignant C2919 Prostate Adenocarcinoma Adenocarcinoma of Prostate || Adenocarcinoma of the Prostate An adenocarcinoma arising from the prostate gland. It is one of the most common malignant tumors afflicting men. The majority of adenocarcinomas arise in the peripheral zone and a minority occurs in the central or the transitional zone of the prostate gland. Grading of prostatic adenocarcinoma predicts disease progression and correlates with survival. Several grading systems have been proposed, of which the Gleason system is the most commonly used. Gleason sums of 2 to 4 represent well-differentiated disease, 5 to 7 moderately differentiated disease and 8 to 10 poorly differentiated disease. Prostatic-specific antigen (PSA) serum test is widely used as a screening test for the early detection of prostatic adenocarcinoma. Treatment options include radical prostatectomy, radiation therapy, androgen ablation and cryotherapy. Watchful waiting or surveillance alone is an option for older patients with low-grade or low-stage disease. Malignant C5539 Prostate Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Prostate || Adenoid Cystic Carcinoma of the Prostate An adenoid cystic carcinoma that arises from the prostate gland. Malignant C4795 Prostate Adenoma Adenoma of Prostate || Adenoma of the Prostate Focal benign glandular hyperplasia in the prostate gland. Benign C5538 Prostate Adenosquamous Carcinoma Adenosquamous Carcinoma of Prostate || Adenosquamous Carcinoma of the Prostate An infrequent invasive carcinoma of the prostate gland characterized by the presence of both glandular and squamous neoplastic components. It is more often located in the transitional zone of the prostate gland and it tends to rapidly metastasize to the bones. Malignant C39902 Prostate Basal Cell Carcinoma Basal Cell Carcinoma of the Prostate An invasive prostate carcinoma composed of prostatic basal cells. Malignant C4863 Prostate Carcinoma Cancer of Prostate || Cancer of the Prostate || Carcinoma of Prostate || Carcinoma of the Prostate || Prostate Cancer One of the most common malignant tumors afflicting men. The majority of carcinomas arise in the peripheral zone and a minority occur in the central or the transitional zone of the prostate gland. Grossly, prostatic carcinomas appear as ill-defined yellow areas of discoloration in the prostate gland lobes. Adenocarcinomas represent the overwhelming majority of prostatic carcinomas. Prostatic-specific antigen (PSA) serum test is widely used as a screening test for the early detection of prostatic carcinoma. Treatment options include radical prostatectomy, radiation therapy, androgen ablation and cryotherapy. Watchful waiting or surveillance alone is an option for older patients with low-grade or low-stage disease. Malignant C6813 Prostate Ductal Adenocarcinoma Ductal Adenocarcinoma of Prostate || Ductal Adenocarcinoma of the Prostate || Endometrioid Adenocarcinoma of Prostate || Endometrioid Adenocarcinoma of the Prostate || Endometrioid Carcinoma of Prostate || Endometrioid Carcinoma of the Prostate || Prostate Endometrioid Adenocarcinoma || Prostate Endometrioid Carcinoma || Prostatic Endometrioid Carcinoma A usually aggressive invasive adenocarcinoma of the prostate gland composed of large glands containing tall columnar cells. The columnar cells have abundant cytoplasm and are reminiscent of endometrial carcinoma. This type of adenocarcinoma has a tendency to metastasize to the lung and penis. Malignant C7574 Prostate Phyllodes Tumor Cystosarcoma Phyllodes of Prostate || Phyllodes Neoplasm of Prostate || Phyllodes Neoplasm of the Prostate || Phyllodes Tumor of Prostate || Phyllodes Tumor of the Prostate || Prostate Cystosarcoma Phyllodes || Prostate Phyllodes Neoplasm An unusual, biphasic benign or malignant neoplasm that arises from the prostate gland. It is characterized by the presence of an epithelial glandular component and a proliferating stroma. Undetermined C6766 Prostate Small Cell Neuroendocrine Carcinoma Prostate Small Cell Carcinoma || Prostate Small Cell NEC || Small Cell Carcinoma of Prostate || Small Cell Carcinoma of the Prostate A rare small cell neuroendocrine carcinoma that arises from the prostate gland. Malignant C5536 Prostate Squamous Cell Carcinoma Squamous Cell Carcinoma of Prostate || Squamous Cell Carcinoma of the Prostate An invasive prostate carcinoma characterized by the presence of malignant cells with squamous differentiation. There is no evidence of glandular differentiation. Malignant C5524 Prostate Stromal Sarcoma Stromal Sarcoma of Prostate || Stromal Sarcoma of the Prostate A rare malignant neoplasm arising from specialized prostatic stroma. It is characterized by the presence of stromal overgrowth and hypercellularity, increased number of mitotic figures, and pleomorphism. Malignant C39905 Prostate Stromal Tumor of Uncertain Malignant Potential Prostate Stromal Proliferation of Uncertain Malignant Potential A neoplastic lesion that arises from the prostate gland stroma. Morphologic characteristics include hypercellular stroma with scattered atypical cells, prominent overgrowth of the prostatic stroma, or changes resembling benign phyllodes tumor. The majority of cases do not have an aggressive clinical course. A minority of cases however, may progress to stromal sarcoma. Uncertain Malignant Potential C5597 Prostate Undifferentiated Carcinoma Undifferentiated Carcinoma of Prostate || Undifferentiated Carcinoma of the Prostate || Undifferentiated Prostate Carcinoma || Undifferentiated Prostatic Carcinoma An invasive prostate carcinoma characterized by the presence of undifferentiated malignant cells. Malignant C4320 Protoplasmic Astrocytoma A rare variant of diffuse astrocytoma. It is predominantly composed of neoplastic astrocytes showing a small cell body with few, flaccid processes with a low content of glial filaments and scant GFAP expression. This lesion is not well defined and is considered by some authors as an occasional histopathological feature rather than a reproducibly identifiable variant. When occurring in children, this neoplasm may be difficult to separate from pilocytic juvenile astrocytoma. (Adapted from WHO) Undetermined C115334 Proximal Urethral Carcinoma A carcinoma that arises from the proximal part of the urethra. Malignant C4331 Psammomatous Meningioma A WHO grade I meningioma characterized by the presence of psammoma bodies that predominate over the meningeal cells. Undetermined C121668 Pseudomyogenic Hemangioendothelioma Pseudomyogenic (Epithelioid Sarcoma-Like) Hemangioendothelioma An intermediate, rarely metastasizing blood vessel neoplasm that more frequently affects young adult males and usually arises in the lower limbs. In approximately half of the affected patients the tumor is painful and in two-thirds of the patients the tumor is multifocal. Morphologically it is characterized by the presence of sheets and fascicles of spindle cells with abundant eosinophilic cytoplasm and vesicular nuclei. Cytologic atypia is usually mild. Approximately 60% of the patients develop local recurrences or additional tumors in the same anatomic region. Undetermined C5381 Pulmonary Artery Choriocarcinoma Choriocarcinoma of Pulmonary Artery || Choriocarcinoma of the Pulmonary Artery || Chorioepithelioma of Pulmonary Artery || Chorioepithelioma of the Pulmonary Artery || Pulmonary Artery Chorioepithelioma || Pulmonary Artery Syncytioma || Syncytioma of Pulmonary Artery || Syncytioma of the Pulmonary Artery A rare choriocarcinoma that arises from a pulmonary artery. Malignant C3732 Pulmonary Blastoma Blastoma of Lung || Blastoma of the Lung || Lung Blastoma || Pneumoblastoma A biphasic tumor that consists of fetal adenocarcinoma (typically low-grade) and primitive mesenchymal stroma. Foci of specific mesenchymal differentiation (osteosarcoma, chondrosarcoma, or rhabdomyosarcoma) may also be present, but are not required for the diagnosis. Most patients are smokers. The prognosis is very poor. (WHO 2015) Malignant C27513 Radiation-Related Angiosarcoma Radiation-Induced Angiosarcoma A malignant vascular neoplasm arising in an anatomic site exposed to radiation therapy. Malignant C53706 Radiation-Related Osteosarcoma Postradiation Osteosarcoma || Radiation-Associated Osteosarcoma A high grade osteosarcoma arising in an irradiated bone. Malignant C9383 Rectal Adenocarcinoma Adenocarcinoma of Rectum || Adenocarcinoma of the Rectum An adenocarcinoma arising from the rectum. It is more frequently seen in populations with a Western type diet and in patients with a history of chronic inflammatory bowel disease. Signs and symptoms include intestinal bleeding, anemia, and change in bowel habits. According to the degree of cellular differentiation, rectal adenocarcinomas are divided into well, moderately, and poorly differentiated. Histologic variants include mucinous adenocarcinoma, signet ring cell carcinoma, medullary carcinoma, serrated adenocarcinoma, cribriform comedo-type adenocarcinoma, and micropapillary adenocarcinoma. Malignant C5546 Rectal Adenoma Adenoma of Rectum || Adenoma of the Rectum An adenoma that arises from the rectum. The group of rectal adenomas includes tubular, villous, and tubulovillous adenomas, traditional serrated adenomas, sessile serrated adenomas/polyps, and familial adenomatous polyposis. Benign C5621 Rectal Adenoma with Severe Dysplasia Adenoma of Rectum with Marked Dysplasia || Adenoma of the Rectum with Marked Dysplasia || Rectal Adenoma with Marked Dysplasia An adenoma that arises from the rectum. It is characterized by the presence of severe epithelial dysplasia. Benign C96480 Rectal Adenomatous Polyp Adenomatous Polyp of Rectum || Adenomatous Polyp of the Rectum A polypoid adenoma that arises from and protrudes into the lumen of the rectum. Epithelial dysplasia is always present. According to the architectural pattern it is classified as tubular, tubulovillous, or villous. Benign C43594 Rectal Adenosquamous Carcinoma An unusual rectal carcinoma characterized by the presence of glandular and squamous carcinomatous components. The two carcinomatous components may be admixed within the tumor, or the two may appear separately in different areas. Malignant C9382 Rectal Carcinoma Cancer of Rectum || Cancer of the Rectum || Carcinoma of Rectum || Carcinoma of the Rectum || Rectal Cancer A malignant epithelial neoplasm that arises from the rectum and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. Malignant C60640 Rectal Medullary Carcinoma A rare, invasive rectal adenocarcinoma characterized by the presence of sheets of malignant epithelial cells with vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It usually has a favorable prognosis. Malignant C7973 Rectal Mucinous Adenocarcinoma Colloid Adenocarcinoma of Rectum || Colloid Adenocarcinoma of the Rectum || Colloidal Adenocarcinoma of Rectum || Colloidal Adenocarcinoma of the Rectum || Mucinous Adenocarcinoma of Rectum || Mucinous Adenocarcinoma of the Rectum || Rectal Colloid Adenocarcinoma || Rectal Colloidal Adenocarcinoma An invasive adenocarcinoma of the rectum characterized by the presence of pools of extracellular mucin. Malignant glandular epithelial cells are present in the mucin collections. Mucin constitutes more than 50% of the lesion. Malignant C96487 Rectal Serrated Adenocarcinoma A rare, invasive rectal adenocarcinoma characterized by the presence of a malignant infiltrate with serrated glandular architecture. Malignant C96465 Rectal Serrated Lesions and Polyps Rectal SSA || Rectal SSA/P || Rectal SSP || Rectal Sessile Serrated Adenoma || Rectal Sessile Serrated Adenoma/Polyp || Rectal Sessile Serrated Polyp Polyps that arises from the rectum. They are characterized by the presence of serrated glands and the absence of generalized dysplasia. Benign C9168 Rectal Signet Ring Cell Adenocarcinoma Rectal Signet-Ring Cell Adenocarcinoma || Signet Ring Adenocarcinoma of Rectum || Signet Ring Adenocarcinoma of the Rectum || Signet Ring Cell Adenocarcinoma of Rectum || Signet Ring Cell Adenocarcinoma of the Rectum An infiltrating adenocarcinoma arising from the rectum. It is characterized by the presence of malignant glandular cells with prominent intracytoplasmic mucin. These cells constitute more than 50% of the malignant cellular population. Malignant C60639 Rectal Small Cell Neuroendocrine Carcinoma Rectal Small Cell Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the rectum. It is characterized by the presence of malignant small cells. Malignant C5554 Rectal Squamous Cell Carcinoma Rectal Squamous Carcinoma || Squamous Carcinoma of Rectum || Squamous Carcinoma of the Rectum || Squamous Cell Carcinoma of Rectum || Squamous Cell Carcinoma of the Rectum A very rare rectal carcinoma characterized by the presence of a malignant squamous cell infiltrate. Malignant C96463 Rectal Traditional Serrated Adenoma Rectal Serrated Adenoma || Rectal Serrated Adenoma Type II || Rectal TSA An adenoma that arises from the rectum. It is characterized by prominent serration of the glands. Benign C96477 Rectal Tubular Adenoma A usually polypoid neoplasm that arises from the glandular epithelium of the rectal mucosa. It is characterized by a tubular architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C5620 Rectal Tubulovillous Adenoma Rectal Villotubular Adenoma || Tubulovillous Adenoma of Rectum || Tubulovillous Adenoma of the Rectum || Villotubular Adenoma of Rectum || Villotubular Adenoma of the Rectum A neoplasm that arises from the glandular epithelium of the rectal mucosa. It is characterized by tubular and villous architectural patterns. The neoplastic glandular cells have dysplastic features. Benign C7974 Rectal Undifferentiated Carcinoma Anaplastic Carcinoma of Rectum || Anaplastic Carcinoma of the Rectum || Carcinoma Simplex of the Rectum. || Rectal Anaplastic Carcinoma || Undifferentiated Carcinoma of Rectum || Undifferentiated Carcinoma of the Rectum || Undifferentiated Rectal Carcinoma An invasive malignant epithelial tumor that arises from the rectum. There is no morphologic, immunophenotypic, or molecular biological evidence of glandular or squamous differentiation. Malignant C4919 Rectal Villous Adenoma Villous Adenoma of Rectum || Villous Adenoma of the Rectum A neoplasm that arises from the glandular epithelium of the rectal mucosa. It is characterized by a villous architectural pattern. The neoplastic glandular cells have dysplastic features. Benign C43584 Rectosigmoid Adenocarcinoma An adenocarcinoma arising from the rectosigmoid area. It is more frequently seen in populations with a Western type diet and in patients with a history of chronic inflammatory bowel disease. Signs and symptoms include intestinal bleeding, anemia, and change in bowel habits. According to the degree of cellular differentiation, rectosigmoid adenocarcinomas are divided into well, moderately, and poorly differentiated. Histologic variants include mucinous adenocarcinoma, signet ring cell carcinoma, medullary carcinoma, serrated adenocarcinoma, cribriform comedo-type adenocarcinoma, and micropapillary adenocarcinoma. Malignant C7421 Rectosigmoid Carcinoma Rectosigmoid Cancer A malignant epithelial neoplasm that arises from the rectosigmoid area and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. About 50% of colorectal carcinomas occur in the rectosigmoid area. Malignant C43592 Rectosigmoid Mucinous Adenocarcinoma An invasive adenocarcinoma of the rectosigmoid area characterized by the presence of pools of extracellular mucin. Malignant glandular epithelial cells are present in the mucin collections. Mucin constitutes more than 50% of the lesion. Malignant C43593 Rectosigmoid Signet Ring Cell Carcinoma Rectosigmoid Signet-Ring Cell Carcinoma An infiltrating adenocarcinoma arising from the rectosigmoid area. It is characterized by the presence of malignant glandular cells with prominent intracytoplasmic mucin. These cells constitute more than 50% of the malignant cellular population. Malignant C2872 Refractory Anemia Aregenerative Anemia A myelodysplastic syndrome characterized mainly by dysplasia of the erythroid series. Refractory anemia is uncommon. It is primarily a disease of older adults. The median survival exceeds 5 years. (WHO, 2001) Malignant C82596 Refractory Cytopenia of Childhood The most common subtype of the myelodysplastic syndromes affecting children. It is characterized by persistent cytopenia with less than 5% blasts in the bone marrow and less than 2% blasts in the peripheral blood. Malignant C82593 Refractory Neutropenia A myelodysplastic syndrome characterized by the presence of at least 10% dysplastic neutrophils in the bone marrow or the peripheral blood. Malignant C82594 Refractory Thrombocytopenia A myelodysplastic syndrome characterized by the presence of at least 10% dysplastic megakaryocytes, found within at least 30 megakaryocytes examined in the bone marrow. Malignant C9385 Renal Cell Carcinoma Adenocarcinoma of Kidney || Adenocarcinoma of the Kidney || Kidney Adenocarcinoma || Renal Cell Adenocarcinoma || Renal Cell Cancer || Renal Cell Carcinoma, Stage Unspecified A carcinoma arising from the renal parenchyma. There is a strong correlation between cigarette smoking and the development of renal cell carcinoma. The clinical presentation includes : hematuria, flank pain and a palpable lumbar mass. A high percentage of renal cell carcinomas are diagnosed when an ultrasound is performed for other purposes. Radical nephrectomy is the standard intervention procedure. Renal cell carcinoma is generally considered to be resistant to radiation treatment and chemotherapy. Malignant C39802 Renal Cell Carcinoma Associated with inv(X)(p11;q12) A renal cell carcinoma with papillary architecture comprised of clear cells. It is characterized by the inv(X)(p11;q12) resulting in fusion of the NONO (p54nrb) and TFE3 genes. Malignant C37874 Renal Cell Carcinoma Associated with t(X;1)(p11.2;p34) A renal cell carcinoma with papillary architecture comprised of clear cells. It is characterized by the t(X;1)(p11.2;p34) resulting in fusion of the PSF and TFE3 genes. Malignant C37872 Renal Cell Carcinoma Associated with t(X;1)(p11.2;q21) A renal cell carcinoma with papillary architecture comprised of clear cells. It is characterized by the t(X;1)(p11.2;q21) resulting in fusion of the PRCC and TFE3 genes. Malignant C37876 Renal Cell Carcinoma Associated with t(X;17)(p11.2;q25) A renal cell carcinoma with papillary architecture comprised of clear cells. It is characterized by the t(X;1)(p11.2;q25) resulting in fusion of the ASPL and TFE3 genes. Malignant C27891 Renal Cell Carcinoma Associated with Xp11.2 Translocations/TFE3 Gene Fusions TFE3-Rearranged Renal Cell Carcinoma || Xp11.2 Translocation-Related Renal Cell Carcinoma A group of renal cell carcinomas characterized by the presence of different translocations involving the chromosome Xp11.2. These translocations result in the creation of gene fusions involving the TFE3 gene. Patients are usually children and young adults. Morphologically, the malignant epithelial cells form papillary patterns. Malignant C6142 Renal Pelvis Carcinoma Carcinoma of Kidney Pelvis || Carcinoma of Renal Pelvis || Carcinoma of the Kidney Pelvis || Carcinoma of the Renal Pelvis || Kidney Pelvis Carcinoma A carcinoma arising in the renal pelvis. The majority of renal pelvis carcinomas are transitional cell and less frequently squamous cell carcinomas. Malignant C6148 Renal Pelvis Papillary Urothelial Carcinoma Kidney Pelvis Papillary Carcinoma || Papillary Carcinoma of Kidney Pelvis || Papillary Carcinoma of Renal Pelvis || Papillary Carcinoma of the Kidney Pelvis || Papillary Carcinoma of the Renal Pelvis A papillary transitional cell carcinoma that arises from the renal pelvis. Malignant C7355 Renal Pelvis Urothelial Carcinoma Renal Pelvis Transitional Cell Carcinoma || Transitional Cell Carcinoma of Renal Pelvis || Transitional Cell Carcinoma of the Renal Pelvis || Urothelial Cell Carcinoma of Renal Pelvis || Urothelial Cell Carcinoma of the Renal Pelvis A carcinoma that arises from the transitional epithelium of the renal pelvis. It is associated with tobacco use and usually presents with gross or microscopic hematuria. Urothelial carcinomas of the renal pelvis are usually of higher grade and higher stage compared to bladder urothelial carcinomas. Malignant C27511 Retiform Hemangioendothelioma Hobnail Hemangioendothelioma An intermediate, locally aggressive and rarely metastasizing blood vessel neoplasm. It is characterized by the presence of hobnail endothelial cells and formation of arborizing vascular channels. Undetermined C7541 Retinoblastoma A malignant tumor that originates in the nuclear layer of the retina. As the most common primary tumor of the eye in children, retinoblastoma is still relatively uncommon, accounting for only 1% of all malignant tumors in pediatric patients. Approximately 95% of cases are diagnosed before age 5. These tumors may be multifocal, bilateral, congenital, inherited, or acquired. Seventy-five percent of retinoblastomas are unilateral; 60% occur sporadically. A predisposition to retinoblastoma has been associated with 13q14 cytogenetic abnormalities. Patients with the inherited form also appear to be at increased risk for secondary nonocular malignancies such as osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. Malignant C66812 Retinocytoma Retinoma A rare, unilateral or bilateral benign neoplasm that arises from the retina. There is an increased risk of malignant transformation to retinoblastoma; therefore, patients with a diagnosis of retinocytoma should be closely observed. Benign C8172 Retromolar Trigone Squamous Cell Carcinoma Squamous Cell Carcinoma of Retromolar Trigone || Squamous Cell Carcinoma of the Retromolar Trigone A squamous cell carcinoma that arises from the retromolar trigone. Pain, often radiating to the ear, is the presenting symptom. The prognosis is poor. Malignant C7352 Retroperitoneal Carcinoma Carcinoma of Retroperitoneum || Carcinoma of the Retroperitoneum A rare carcinoma that arises from the retroperitoneal space. Malignant C6909 Rhabdoid Meningioma A WHO grade III meningioma characterized by the predominant presence of rhabdoid cells forming sheets. Malignant C3808 Rhabdoid Tumor An aggressive malignant embryonal neoplasm usually occurring during childhood. It is characterized by the presence of large cells with abundant cytoplasm, large eccentric nucleus, and a prominent nucleolus and it is associated with abnormalities of chromosome 22. It can arise from the central nervous system, kidney, and the soft tissues. The prognosis is poor. Malignant C8715 Rhabdoid Tumor of the Kidney Kidney Rhabdoid Tumor || Malignant Rhabdoid Tumor of Kidney || Malignant Rhabdoid Tumor of the Kidney || Renal Rhabdoid Neoplasm || Renal Rhabdoid Tumor || Rhabdoid Neoplasm of Kidney || Rhabdoid Neoplasm of the Kidney || Rhabdoid Tumor of the Kidney (RTK) || Rhabdoid Tumour of Kidney || Rhabdoid Tumour of the Kidney A rhabdoid tumor that arises from the kidney. It occurs in children and it is associated with abnormalities of chromosome 22. It is characterized by the presence of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. The prognosis is poor. Malignant C3358 Rhabdomyoma A benign mesenchymal tumor arising from skeletal or cardiac muscle. Benign C3359 Rhabdomyosarcoma A rare aggressive malignant mesenchymal neoplasm arising from skeletal muscle. It usually occurs in children and young adults. Only a small percentage of tumors arise in the skeletal muscle of the extremities. The majority arise in other anatomic sites. Malignant C4259 Rhabdomyosarcoma with Mixed Embryonal and Alveolar Features Mixed Alveolar Rhabdomyosarcoma || Mixed Type Alveolar Rhabdomyosarcoma A rhabdomyosarcoma composed of embryonic and alveolar components. It is characterized by the presence of spindle cells with myoblastic differentiation, a myxoid stroma, and fibrous septa. These tumors were previously considered variants of alveolar rhabdomyosarcoma. The lack of PAX3-FOXO1 fusions in most of these tumors suggests that are biologically and clinically related to embryonal rhabdomyosarcoma. Malignant C67559 Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle A central nervous system neoplasm arising from the fourth ventricle. It is characterized by the presence of neurocytes forming pseudorosettes and astrocytes which contain Rosenthal fibers. Cytologic atypia is minimal. Undetermined C8021 Salivary Gland Adenocarcinoma Adenocarcinoma of Salivary Gland || Adenocarcinoma of the Salivary Gland An adenocarcinoma arising from the salivary gland. It includes the salivary gland polymorphous low grade adenocarcinoma, salivary gland oncocytic carcinoma, salivary gland mucinous adenocarcinoma, salivary gland low grade cribriform cystadenocarcinoma, salivary gland cystadenocarcinoma, salivary gland basal cell adenocarcinoma, salivary gland acinic cell carcinoma, salivary duct carcinoma, and salivary gland adenocarcinoma not otherwise specified. Malignant C8026 Salivary Gland Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Salivary Gland || Adenoid Cystic Carcinoma of the Salivary Gland An adenoid cystic carcinoma arising from the salivary gland. It is characterized by the presence of epithelial and myoepithelial cells forming tubular, cribriform, and solid patterns. It usually presents as a slow growing mass. Patients may experience pain because of the tumor propensity for perineural invasion. The tumor may follow an aggressive clinical course with recurrences and mestastases to distant sites including lungs, bones, brain, and liver. Malignant C35737 Salivary Gland Adenosquamous Carcinoma A rare, aggressive carcinoma that arises from the salivary glands. It is characterized by the presence of a squamous and a glandular epithelial component. Malignant C9272 Salivary Gland Carcinoma Carcinoma of Salivary Gland || Carcinoma of the Salivary Gland A carcinoma that arises from the major or minor salivary glands. Representative examples include carcinoma ex pleomorphic adenoma, adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma. Malignant C40410 Salivary Gland Carcinoma ex Pleomorphic Adenoma A carcinoma that arises from a pleomorphic adenoma in the salivary glands. It usually originates in the parotid gland. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive. Malignant C62191 Salivary Gland Clear Cell Carcinoma Salivary Gland Clear Cell Carcinoma, NOS || Salivary Gland Clear Cell Carcinoma, Not Otherwise Specified A carcinoma that arises from the salivary glands, most often the minor salivary glands. It is characterized by the presence of a monomorphic population of malignant epithelial cells with clear cytoplasm and the absence of morphologic features that define other primary neoplasms of the salivary glands. Malignant C35701 Salivary Gland Epithelial-Myoepithelial Carcinoma Salivary Gland Epithelial Myoepithelial Carcinoma A carcinoma that arises from the salivary glands, most often the parotid gland. It presents as a slow growing and painless mass. It is characterized by the presence of duct-like structures lined by two layers of cells, an inner layer composed of epithelial-type cells and an outer layer composed of clear, myoepithelial-type cells. Malignant C35736 Salivary Gland Lymphoepithelial Carcinoma Lymphoepithelial Salivary Gland Carcinoma A rare carcinoma that arises from the salivary glands, most often the parotid gland. It is characterized by the presence of an undifferentiated carcinomatous component associated with a prominent reactive lymphoplasmacytic infiltrate. Malignant C3686 Salivary Gland Monomorphic Adenoma A benign epithelial neoplasm arising from the salivary glands. It is characterized by the presence of a monomorphic cellular infiltrate. Benign C5908 Salivary Gland Mucoepidermoid Carcinoma Mucoepidermoid Carcinoma of Salivary Gland || Mucoepidermoid Carcinoma of the Salivary Gland A carcinoma that arises from the salivary glands. It is the most common primary carcinoma of the salivary glands and usually presents as a firm and painless mass. It is characterized by the presence of squamous cells, mucus producing cells, and cells of intermediate type. The majority of cases have a favorable outcome. Malignant C35700 Salivary Gland Myoepithelial Carcinoma A locally aggressive carcinoma that arises from the salivary glands, predominantly the parotid gland. It is characterized by the presence of a malignant cellular infiltrate exhibiting myoepithelial differentiation. Patients usually present with a painless mass. Malignant C40409 Salivary Gland Pleomorphic Adenoma Benign Mixed Salivary Gland Neoplasm || Benign Mixed Salivary Gland Tumor || Benign Mixed Salivary Neoplasm || Benign Mixed Salivary Tumor A benign, slow-growing tumor composed of cells that demonstrate both epithelial and mesenchymal differentiation. It is the most common neoplasm of salivary gland origin, and mostly occurs in the parotid gland. The average age of patients has been reported to be 43 years; women are more often affected than men. The malignant change rate has been estimated to be 6%. The malignant counterpart is carcinoma ex pleomorphic adenoma. Benign C35703 Salivary Gland Small Cell Neuroendocrine Carcinoma An infrequent small cell carcinoma that arises from the salivary glands. It is characterized by the presence of neuroendocrine differentiation and a high number of mitotic figures. Malignant C7991 Salivary Gland Squamous Cell Carcinoma Epidermoid Carcinoma of Salivary Gland || Epidermoid Carcinoma of the Salivary Gland || SCC of Salivary Gland || SCC of the Salivary Gland || Salivary Gland Epidermoid Carcinoma || Salivary Gland SCC || Squamous Cell Carcinoma of Salivary Gland || Squamous Cell Carcinoma of the Salivary Gland A squamous cell carcinoma arising from the salivary glands. The majority of patients are in their sixth through eight decades. It usually presents as a rapidly enlarging mass, which may be painful. It usually has an aggressive clinical course. Malignant C8024 Salivary Gland Undifferentiated Carcinoma Anaplastic Carcinoma of Salivary Gland || Anaplastic Carcinoma of the Salivary Gland || Anaplastic Salivary Gland Carcinoma || Salivary Gland Anaplastic Carcinoma || Undifferentiated Carcinoma of Salivary Gland || Undifferentiated Carcinoma of the Salivary Gland || Undifferentiated Salivary Gland Carcinoma A salivary gland carcinoma characterized by the presence of undifferentiated, anaplastic malignant epithelial cells. Malignant C9118 Sarcoma Sarcoma of Soft Tissue and Bone || Sarcoma of the Soft Tissue and Bone A usually aggressive malignant neoplasm of the soft tissue or bone. It arises from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The prognosis depends largely on the degree of differentiation (grade) of the neoplasm. Representative subtypes are liposarcoma, leiomyosarcoma, osteosarcoma, and chondrosarcoma. Malignant C27004 Sarcomatoid Carcinoma Pseudosarcomatous Carcinoma || Spindle Cell Carcinoma A malignant epithelial neoplasm characterized by the presence of spindle cells and anaplastic morphologic features. Giant cells and a sarcomatous component may also be present. Malignant C45655 Sarcomatoid Mesothelioma A malignant neoplasm arising from mesothelial cells. It is characterized by the presence of spindle cells. Anaplastic morphologic features and multinucleated malignant cells may also be seen. Malignant C27893 Sarcomatoid Renal Cell Carcinoma A high grade carcinoma of the kidney. It is not a distinct clinicopathological entity and includes a diverse group of renal cell carcinomas which have been transformed from a lower to a higher grade. Malignant C3269 Schwannoma Benign Neurilemmoma || Benign Schwannoma || Neurilemmoma || Schwannoma (WHO Grade I) A benign, usually encapsulated slow growing tumor composed of Schwann cells. It affects peripheral and cranial nerves. It recurs infrequently and only rare cases associated with malignant transformation have been reported. Benign C121655 Sclerosing Rhabdomyosarcoma An uncommon variant of rhabdomyosarcoma with sclerosing morphology. It usually arises from the limbs. Malignant C4174 Sebaceous Adenoma Adenoma of Sebaceous Gland || Adenoma of the Sebaceous Gland || Sebaceous Gland Adenoma || Skin Appendage Sebaceous Adenoma A benign, well circumscribed neoplasm arising from the sebaceous glands. It usually presents as a small yellowish tumor in the sun exposed skin of head and neck. It is characterized by the presence of sebaceous cells aggregates with a peripheral rim of basaloid cells. Benign C40310 Sebaceous Carcinoma Carcinoma of Sebaceous Gland || Carcinoma of the Sebaceous Gland || Sebaceous Gland Carcinoma An adenocarcinoma with sebaceous differentiation. It presents as a painless mass and it may be multifocal. It grows in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. It is characterized by the presence of malignant cells with multivesicular and clear cytoplasm. It may recur and metastasize. Malignant C25765 Secondary Acute Myeloid Leukemia Secondary AGL || Secondary AML || Secondary Acute Myeloblastic Leukemia || Secondary Acute Myelogenous Leukemia An acute myeloid leukemia arising from prior myelodysplastic syndrome, myeloproliferative neoplasm, or myelodysplastic/myeloproliferative neoplasm, or as a result of previous exposure to alkylating agents or topoisomerase II inhibitors. Malignant C27482 Secondary Chondrosarcoma A chondrosarcoma that arises either in a pre-existing enchondroma or within the cartilaginous cap of a pre-existing osteochondroma. Malignant C39751 Secondary Glioblastoma Secondary Glioblastoma Multiforme A glioblastoma arising from a lower grade astrocytoma. It is more commonly seen in younger patients and is usually associated with IDH1 or IDH2 gene mutations. Malignant C53704 Secondary Osteosarcoma Secondary Bone Osteosarcoma An osteosarcoma arising from a pre-existing lesion of the bone, usually Paget disease, or due to radiation therapy. Malignant C4718 Secretory Meningioma A WHO grade I meningioma characterized by the presence of epithelial differentiation and numerous intracellular PAS positive bodies that are rich in glycogen. Undetermined C9309 Seminoma Seminoma, Pure A radiosensitive malignant germ cell tumor found in the testis (especially undescended), and extragonadal sites (anterior mediastinum and pineal gland). It is characterized by the presence of uniform cells with clear or dense cytoplasm which contains glycogen, and by a large nucleus which contains one or more nucleoli. The neoplastic germ cells form aggregates separated by fibrous septa. The fibrous septa contain chronic inflammatory cells, mainly lymphocytes. Malignant C40101 Serous Adenocarcinoma An adenocarcinoma that is characterized by the presence of papillary patterns and cellular budding. Psammoma bodies may be present. Representative examples include cervical serous adenocarcinoma, endometrial serous adenocarcinoma, ovarian serous adenocarcinoma, and primary peritoneal serous adenocarcinoma. Malignant C40152 Serous Endometrial Intraepithelial Carcinoma Endometrial Intraepithelial Carcinoma || Endometrial Serous Carcinoma in Situ || Endometrial Serous Intraepithelial Carcinoma || Endometrial Surface Serous Carcinoma A precursor of endometrial serous adenocarcinoma characterized by the replacement of the normal endometrial cells by malignant glandular cells that resemble the malignant cells of invasive serous adenocarcinoma. The endometrial neoplastic process is non-invasive. It may coexist with invasive endometrial adenocarcinoma or can be associated with metastases outside the uterus, in the absence of invasive endometrial carcinoma. Malignant C3366 Sezary Syndrome Sezary's Disease || Sézary Syndrome A generalized peripheral (mature) T-cell neoplasm characterized by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sezary syndrome is an aggressive disease. (WHO, 2001) Malignant C3774 Signet Ring Cell Carcinoma Signet Ring Cell Adenocarcinoma || Signet-Ring Cell Carcinoma A usually aggressive, poorly differentiated invasive adenocarcinoma characterized by the presence of malignant glandular cells in which the nucleus is pressed to one side by the presence of intracytoplasmic mucus. It may arise from the stomach, small and large intestine, ampulla of Vater, appendix, gallbladder, pancreas, lung, bladder, breast, and prostate gland. Malignant C68611 Sinonasal Squamous Cell Carcinoma Nasal Cavity and Paranasal Sinus Squamous Cell Carcinoma A rare, keratinizing or non-keratinizing squamous cell carcinoma arising from the mucosal epithelium of the nasal cavity or the paranasal sinuses. It affects most often the maxillary sinus. Less frequently, it arises from the nasal cavity, ethmoid sinus, sphenoid sinus, and frontal sinus. Symptoms include nasal fullness, epistaxis, rhinorhea, pain, and paresthesia. Patients with nasal squamous cell carcinoma usually present earlier than patients with maxillary sinus carcinoma and have a better prognosis compared to the latter group. Malignant C54250 Skin Adenosquamous Carcinoma An uncommon carcinoma that arises from the skin. It is characterized by the presence of malignant glandular and malignant squamous cellular components. Malignant C4489 Skin Angiosarcoma Angiosarcoma of Skin || Angiosarcoma of the Skin || Hemangiosarcoma of Skin || Hemangiosarcoma of the Skin || Skin Hemangiosarcoma A malignant vascular neoplasm arising from the skin. Malignant C2921 Skin Basal Cell Carcinoma Basal Cell Cancer || Basal Cell Carcinoma || Basal Cell Carcinoma of Skin || Basal Cell Carcinoma of the Skin || Basal Cell Epithelioma || Basal Cell Skin Carcinoma The most frequently seen skin cancer. It arises from basal cells of the epidermis and pilosebaceous units. Clinically it is divided into the following types: nodular, ulcerative, superficial, multicentric, erythematous, and sclerosing or morphea-like. More than 95% of these carcinomas occur in patients over 40. They develop on hair-bearing skin, most commonly on sun-exposed areas. Approximately 85% are found on the head and neck and the remaining 15% on the trunk and extremities. Basal cell carcinoma usually grows in a slow and indolent fashion. However, if untreated, the tumor may invade the subcutaneous fat, skeletal muscle and bone. Distant metastases are rare. Excision, curettage and irradiation cure most basal cell carcinomas. Malignant C4914 Skin Carcinoma Carcinoma of Skin || Carcinoma of the Skin || Non-Melanoma Cancer of Skin || Non-Melanoma Cancer of the Skin || Non-Melanoma Skin Cancer || Skin Cancer, Non-Melanoma A carcinoma that arises from the skin. Representative examples are basal cell carcinoma and squamous cell carcinoma. Malignant C6801 Skin Fibrous Histiocytoma Benign Cutaneous Fibrous Histiocytoma || Benign Fibrous Cutaneous Histiocytoma || Benign Fibrous Histiocytoma of Skin || Benign Fibrous Histiocytoma of the Skin || Benign Skin Fibrous Histiocytoma || Cutaneous Fibrous Histiocytoma || Dermatofibroma || Fibrous Histiocytoma of Skin || Fibrous Histiocytoma of the Skin A solitary, slowly growing, nodular tumor most often affecting the extremities. It is composed of fibrous and histiocytic cells which infiltrate the dermis and occasionally the underlying subcutaneous tissue. Usually local excision is curative. Recurrences are reported only in a small minority of cases. Benign C4905 Skin Hemangioma Angioma of Skin || Angioma of the Skin || Hemangioma of Skin || Hemangioma of the Skin || Skin Angioma A hemangioma arising from the skin. Benign C27509 Skin Lymphangioma Cutaneous Lymphangioma A lymphangioma arising from the skin. Benign C4614 Skin Papilloma Cutaneous Papilloma || Papilloma of Skin || Papilloma of the Skin A benign papillary neoplastic growth on the skin composed of epithelial cells and a fibrous stalk. It usually develops in the eyelid, axilla, neck, upper chest, and groin. Benign C4819 Skin Squamous Cell Carcinoma Cutaneous Squamous Cell Carcinoma || Epidermoid Carcinoma of Skin || Epidermoid Carcinoma of the Skin || Epidermoid Skin Carcinoma || Squamous Cell Carcinoma of Skin || Squamous Cell Carcinoma of the Skin || Squamous Cell Skin Carcinoma A carcinoma arising from the squamous cells of the epidermis. Skin squamous cell carcinoma is most commonly found on sun-exposed areas. The majority of the tumors are well-differentiated. Malignant C5453 Skull Base Chordoma Chordoma of Skull Base || Chordoma of the Skull Base A chordoma that arises from the base of the skull. Malignant C125890 Small Cell Glioblastoma A morphologic variant of glioblastoma characterized by the presence of a highly proliferative and monotonous population of malignant small glial cells. Malignant C3915 Small Cell Neuroendocrine Carcinoma Small Cell Cancer || Small Cell Carcinoma || Small Cell NEC An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation. It is composed of malignant small cells. Malignant C7208 Small Cell Variant Anaplastic Large Cell Lymphoma A histologic variant of anaplastic large cell lymphoma characterized by the presence of a predominant population of small to medium size malignant cells with irregular nuclei. Malignant C7888 Small Intestinal Adenocarcinoma Adenocarcinoma of Small Bowel || Adenocarcinoma of Small Intestine || Adenocarcinoma of the Small Bowel || Adenocarcinoma of the Small Intestine || Non-Ampullary Adenocarcinoma || Small Bowel Adenocarcinoma An adenocarcinoma that arises from the small intestine. Histologic variants include mucinous adenocarcinoma and signet ring cell carcinoma. Malignant C7724 Small Intestinal Carcinoma Cancer of Small Bowel || Cancer of the Small Bowel || Carcinoma of Small Bowel || Carcinoma of Small Intestine || Carcinoma of the Small Bowel || Carcinoma of the Small Intestine || Small Bowel Cancer || Small Bowel Carcinoma || Small Intestine Cancer A carcinoma arising from the small intestine. The vast majority are adenocarcinomas. The remaining cases are adenosquamous, squamous, or undifferentiated carcinomas. Malignant C43537 Small Intestinal Medullary Carcinoma A carcinoma that arises from the small intestine. It is characterized by the presence of malignant epithelial cells with vesicular nucleus, distinct nucleolus, and abundant pink cytoplasm. Malignant C43536 Small Intestinal Mucinous Adenocarcinoma An invasive adenocarcinoma that arises from the small intestine. It is composed of malignant glandular cells which contain intracytoplasmic mucin. Often, the infiltrating glandular structures are associated with mucoid stromal formation. Malignant C43543 Small Intestinal Signet Ring Cell Carcinoma Small Intestinal Signet-Ring Cell Carcinoma An invasive adenocarcinoma that arises from the small intestine. It is characterized by the presence of malignant glandular cells in which the nucleus is pressed to one side by the presence of intracytoplasmic mucus. Malignant C27449 Small Intestinal Small Cell Neuroendocrine Carcinoma Small Intestinal Small Cell Carcinoma An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the small intestine. It is characterized by the presence of malignant small cells. Malignant C7540 Small Lymphocytic Lymphoma B-Cell Small Lymphocytic Lymphoma || Small B-Cell Lymphocytic Lymphoma A non-Hodgkin lymphoma composed of monomorphic small, round B-lymphocytes in the lymph nodes. When the lymphoid process predominantly involves the bone marrow and the peripheral blood it is called chronic lymphocytic leukemia. (WHO, 2001) Malignant C37203 Small Lymphocytic Lymphoma with Immunoglobulin Heavy Chain Variable-Region Gene Somatic Hypermutation Postgerminal Center Small Lymphocytic Lymphoma Small lymphocytic lymphoma characterized by the presence of somatic hypermutation within the immunoglobulin heavy chain gene variable region of neoplastic clones. Patients have a better prognosis as compared to those with unmutated immunoglobulin heavy chain gene variable region rearrangements. Malignant C36273 Small Lymphocytic Lymphoma with Plasmacytoid Differentiation A small lymphocytic lymphoma characterized by the presence of neoplastic lymphocytes with plasmacytoid morphology. Malignant C37206 Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene Pregerminal Center Small Lymphocytic Lymphoma Small lymphocytic lymphoma characterized by the absence of somatic hypermutation within the immunoglobulin heavy chain gene variable region of neoplastic clones. Patients have a significantly worse prognosis as compared to those with mutated immunoglobulin heavy chain gene variable region rearrangements. Malignant C7149 Smoldering Plasma Cell Myeloma Asymptomatic Myeloma || Asymptomatic Plasma Cell Myeloma || Smoldering Multiple Myeloma || Smoldering Myeloma A plasma cell myeloma lacking clinical manifestations and organ impairment. Malignant C5651 Solid Lung Adenocarcinoma Lung Solid Adenocarcinoma with Mucin || Pulmonary Solid Adenocarcinoma with Mucin || Solid Adenocarcinoma of Lung with Mucin || Solid Adenocarcinoma of the Lung with Mucin A morphologic variant of lung adenocarcinoma characterized by the presence of polygonal malignant cells forming sheets. Acinar, papillary, micropapillary, or lepidic growth patterns are absent. If the tumor is 100 percent solid, intracellular mucin should be present in at least five tumor cells in each of two high-power fields and confirmed with mucin immunohistochemical stains. Tumors formerly classified as large cell carcinomas expressing pneumocyte immunohistochemical markers, even if intracellular mucin is absent, are now included in this category. Malignant C121681 Solitary Circumscribed Neuroma Palisaded Encapsulated Neuroma A benign peripheral nerve sheath tumor characterized by the presence of Schwann cells, axons, and perineurial fibroblasts. It usually arises from the skin of the head and neck or the oral mucosa. It presents as a solitary and painless nodular mass. Benign C7634 Solitary Fibrous Tumor Localized Fibrous Tumor || Solitary Fibrous Tumor, NOS || Solitary Fibrous Tumor, Not Otherwise Specified A localized neoplasm of probable fibroblastic derivation. It is characterized by the presence of round to spindle-shaped cells, hylanized stroma formation, thin-walled branching blood vessels, and thin bands of collagen. Undetermined C6932 Solitary Plasmacytoma A localized clonal (malignant) plasma cell infiltrate either in the bony skeleton or soft tissue without peripheral blood involvement. Malignant C7812 Solitary Plasmacytoma of Bone Isolated Osseous Plasmacytoma || Isolated Plasmacytoma of Bone A localized, clonal (malignant) plasma cell infiltrate in the bone, without peripheral blood involvement. The most commonly affected bones are the vertebrae, ribs, skull, pelvis and femur. X-rays examination reveals a solitary lytic lesion. Malignant C7461 Somatotroph Adenoma GH Cell Adenoma || Growth Hormone Producing Adenoma of Pituitary || Growth Hormone Producing Adenoma of Pituitary Gland || Growth Hormone Producing Adenoma of the Pituitary || Growth Hormone Producing Adenoma of the Pituitary Gland || Growth Hormone Producing Pituitary Adenoma || Growth Hormone Producing Pituitary Gland Adenoma || Growth Hormone Secreting Adenoma of Pituitary || Growth Hormone Secreting Adenoma of Pituitary Gland || Growth Hormone Secreting Adenoma of the Pituitary || Growth Hormone Secreting Adenoma of the Pituitary Gland || Growth Hormone Secreting Pituitary Adenoma || Growth Hormone Secreting Pituitary Gland Adenoma || Growth Hormone-Producing Pituitary Gland Adenoma || Somatotrope Adenoma || Somatotrophinoma An adenoma of the anterior lobe of the pituitary gland that produces growth hormone. The vast majority of cases are hormonally functioning and are associated with either gigantism or acromegaly. Benign C3381 Spinal Cord Neoplasm Neoplasm of Spinal Cord || Neoplasm of the Spinal Cord || Spinal Cord Tumor || Tumor of Spinal Cord || Tumor of the Spinal Cord A primary or metastatic neoplasm affecting the spinal cord. Undetermined C4237 Spindle Cell Melanoma Malignant Spindle Cell Melanoma || Spindle Cell Malignant Melanoma A melanoma characterized by the presence of malignant spindle-shaped melanocytes. Malignant C6519 Spindle Cell Rhabdomyosarcoma An uncommon variant of rhabdomyosarcoma characterized by the presence of whorls of spindle cells forming a storiform pattern. In children it usually arises in the paratesticular region. In adults it usually arises from the deep soft tissues in the head and neck. Malignant C5117 Spiradenocarcinoma Carcinoma ex Eccrine Spiradenoma || Malignant Eccrine Spiradenoma || Malignant Spiradenoma A very rare, aggressive carcinoma of the sweat glands arising from malignant transformation of a long standing spiradenoma. It usually grows in the upper extremities, lower extremities, trunk, and head and neck. It has the tendency to recur and metastasize most often to the lymph nodes, bones, and lungs. Malignant C4170 Spiradenoma Benign Eccrine Spiradenoma || Eccrine Spiradenoma A benign epithelial neoplasm with eccrine or apocrine differentiation, arising from the sweat glands. It usually presents as a solitary, well circumscribed, firm nodule in the face and upper trunk. It is characterized by the presence of basaloid cells forming nodules in the dermis. Cases of carcinoma arising from long standing spiradenomas have been reported. Benign C27007 Spitz Nevus Benign Juvenile Melanoma || Juvenile Nevus || Spindle and/ or Epithelioid Cell Nevus A benign, acquired or congenital, usually single skin lesion. It can occur on any area of the body, but most commonly occurs on the face of children and the thighs of young females. It is characterized by a proliferation of large spindle, oval, or large epithelioid melanocytes in the dermal-epidermal junction. The melanocytic proliferation subsequently extends into the dermis. Benign C80308 Splenic B-Cell Lymphoma/Leukemia, Unclassifiable A small B-cell clonal lymphoproliferative disorder of the spleen that does not fall into any of the other categories of mature B-cell neoplasms. Malignant C80309 Splenic Diffuse Red Pulp Small B-Cell Lymphoma An uncommon, indolent B-cell non-Hodgkin lymphoma composed of small B-lymphocytes involving the red pulp of the spleen, bone marrow, and peripheral blood. Patients often have massive splenomegaly. The peripheral blood examination reveals villous lymphocytes. Malignant C4663 Splenic Marginal Zone Lymphoma Marginal Zone Lymphoma of Spleen || Marginal Zone Lymphoma of the Spleen || Splenic Lymphoma with Circulating Villous Lymphocytes || Splenic Marginal Zone B-Cell Lymphoma || Splenic Marginal Zone B-Cell Lymphoma with Villous Lymphocytes || Splenic Marginal Zone Lymphoma with Villous Lymphocytes A B-cell non-Hodgkin lymphoma composed of small lymphocytes which surround and replace the splenic white pulp germinal centers. It involves the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood. When lymphoma cells are present in the peripheral blood, they are usually, but not always, characterized by the presence of short polar villi. (WHO) Malignant C7566 Sporadic Breast Carcinoma Sporadic Breast Cancer A carcinoma that arises from the breast and is not caused by inherited genetic mutations. Malignant C43529 Sporadic Gastric Diffuse Adenocarcinoma Sporadic Diffuse Gastric Adenocarcinoma A sporadic adenocarcinoma that arises from the gastric mucosa and is characterized by the presence of poorly cohesive malignant cells and absence of glandular formations. Malignant C27890 Sporadic Papillary Renal Cell Carcinoma A papillary renal cell carcinoma that occurs in a patient who does not have a family history of papillary renal cell carcinoma nor is a carrier of an inherited DNA change that would increase the risk of developing this carcinoma. Malignant C42596 Sporadic Retinoblastoma A retinoblastoma that occurs in a patient without a family history of the disease. Malignant C46098 Sporadic Thyroid Gland Medullary Carcinoma A non-hereditary medullary carcinoma of the thyroid gland not associated with multiple endocrine neoplasia. The majority of thyroid gland medullary carcinomas are sporadic. Malignant C2929 Squamous Cell Carcinoma Epidermoid Carcinoma || Epidermoid Cell Cancer || Malignant Epidermoid Cell Neoplasm || Malignant Epidermoid Cell Tumor || Malignant Squamous Cell Neoplasm || Malignant Squamous Cell Tumor || Squamous Carcinoma || Squamous Cell Cancer || Squamous Cell Carcinoma, NOS || Squamous Cell Carcinoma, Not Otherwise Specified || Squamous Cell Epithelioma A carcinoma arising from squamous epithelial cells. Morphologically, it is characterized by the proliferation of atypical, often pleomorphic squamous cells. Squamous cell carcinomas are graded by the degree of cellular differentiation as well, moderately, or poorly differentiated. Well differentiated carcinomas are usually associated with keratin production and the presence of intercellular bridges between adjacent cells. Representative examples are lung squamous cell carcinoma, skin squamous cell carcinoma, and cervical squamous cell carcinoma. Malignant C7729 Squamous Cell Carcinoma of the Penis Epidermoid Carcinoma of Penis || Epidermoid Carcinoma of the Penis || Epidermoid Cell Carcinoma of Penis || Epidermoid Cell Carcinoma of the Penis || Penile Epidermoid Carcinoma || Penile Epidermoid Cell Carcinoma || Penile Squamous Cell Carcinoma || Squamous Cell Carcinoma of Penis A squamous cell carcinoma arising from the penis. It occurs chiefly in the squamous epithelium of the glans, coronal sulcus, and foreskin. Etiologic factors include phimosis, lichen sclerosus, smoking, ultraviolet irradiation, history of warts or condylomas, and lack of circumcision. Human papilloma virus is present in a subset of penile squamous cell carcinomas. Patients may present with an exophytic or flat ulcerative mass in the glans or a large primary tumor with inguinal nodal and skin metastases. (WHO, 2004) Malignant C3712 Squamous Cell Papilloma Epidermoid Cell Papilloma || Epidermoid Papilloma || Keratotic Papilloma || Squamous Papilloma A benign epithelial neoplasm characterized by a papillary growth pattern and a proliferation of neoplastic squamous cells without morphologic evidence of malignancy. Most frequently it arises in the oral cavity, nasopharynx, larynx, esophagus, vagina, and vulva. Benign C4444 Stage 0 Ampulla of Vater Cancer AJCC v7 Ampulla of Vater Cancer Stage 0 || Ampulla of Vater Carcinoma in situ || Ampullary Carcinoma in Situ || Stage 0 Ampulla of Vater Cancer || Stage 0 Ampulla of Vater Carcinoma || Stage 0 Ampullary Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (from AJCC 7th Ed.) Malignant C7794 Stage 0 Anal Canal Cancer AJCC v6 and v7 Anal Canal Carcinoma in situ || Stage 0 Anal Canal Cancer || Stage 0 Anal Canal Cancer AJCC v6 || Stage 0 Anal Canal Cancer AJCC v7 || Stage 0 Anal Canal Carcinoma AJCC v6 and v7 || Stage 0 Anal Carcinoma AJCC v6 and v7 Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th Eds.) Malignant C4593 Stage 0 Appendix Carcinoma AJCC v7 Carcinoma in situ of Appendix || Carcinoma in situ of the Appendix || Stage 0 Appendix Cancer || Stage 0 Appendix Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial or invasion of lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C6188 Stage 0 Bladder Urothelial Carcinoma AJCC v6 and v7 Non-Invasive Bladder Urothelial Carcinoma || Stage 0 Bladder Urothelial Carcinoma || Stage 0 Bladder Urothelial Carcinoma AJCC v7 || Stage 0 Transitional Cell Carcinoma of Bladder || Stage 0 Transitional Cell Carcinoma of Urinary Bladder || Stage 0 Transitional Cell Carcinoma of the Bladder || Stage 0 Transitional Cell Carcinoma of the Urinary Bladder || Stage 0 Urinary Bladder Transitional Cell Carcinoma Stage 0 includes: 0a (Ta, N0, M0); 0is (Tis, N0, M0). Ta: Noninvasive papillary carcinoma. Tis: Carcinoma in situ: "flat tumor". N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C89550 Stage 0 Cervical Cancer AJCC v7 CIN Grade 3 || CIN Grade 3 AJCC v7 || Carcinoma in situ of the Cervix || Carcinoma in situ of the Cervix AJCC v7 || Cervical Carcinoma in situ || Cervical Carcinoma in situ AJCC v7 || Cervix Intraepithelial Neoplasia Grade 3 || Cervix Intraepithelial Neoplasia Grade 3 AJCC v7 || Severe Cervical Dysplasia || Severe Cervical Dysplasia AJCC v7 || Severe Dysplasia of Uterine Cervix || Severe Dysplasia of Uterine Cervix AJCC v7 || Stage 0 Cervical Cancer Stage 0 includes: (Tis, N0, M0). Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. FIGO no longer includes stage 0. (AJCC 7th ed.) Malignant C3638 Stage 0 Colon Cancer AJCC v6 and v7 Carcinoma in situ of Colon || Carcinoma in situ of the Colon || Colon Cancer Stage 0 || Colon Carcinoma in situ || Colonic Carcinoma in situ || Stage 0 Carcinoma of Colon || Stage 0 Carcinoma of the Colon || Stage 0 Colon Cancer AJCC v6 || Stage 0 Colon Cancer AJCC v7 || Stage 0 Colon Carcinoma || Stage 0 Colonic Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial or invasion of lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C8932 Stage 0 Colorectal Cancer AJCC v6 and v7 Colorectal Cancer, Stage 0 || Colorectal Carcinoma Stage 0 || Colorectal Carcinoma in situ || Stage 0 Colorectal Cancer AJCC v6 || Stage 0 Colorectal Cancer AJCC v7 || Stage 0 Colorectal Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial or invasion of the lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C8629 Stage 0 Esophageal Adenocarcinoma AJCC v7 Adenocarcinoma in situ of Esophagus || Adenocarcinoma in situ of the Esophagus || Esophageal Adenocarcinoma in situ || Esophageal Adenocarcinoma, Stage 0 || Esophagus Adenocarcinoma in situ || Oesophageal Adenocarcinoma Stage 0 || Stage 0 Adenocarcinoma of Esophagus || Stage 0 Adenocarcinoma of the Esophagus || Stage 0 Esophageal Adenocarcinoma || Stage 0 Esophagus Adenocarcinoma Stage 0 includes: Tis (HGD), N0, M0, G1,GX. Tis: High-grade dysplasia. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well differentiated. GX: Grade cannot be assessed-stage grouping as G1. (AJCC 7th ed.) Malignant C89771 Stage 0 Esophageal Cancer AJCC v7 Esophageal Carcinoma in situ || Esophageal Carcinoma in situ AJCC v7 || Severe Esophageal Dysplasia || Severe Esophageal Dysplasia AJCC v7 || Stage 0 Esophageal Cancer Stage 0 includes: For squamous cell carcinoma: Tis (HGD), N0, M0, G1, GX, Tumor location: Any. For adenocarcinoma: Tis (HGD), N0, M0, G1, GX. Tis: High-grade dysplasia. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well differentiated. GX: Grade cannot be assessed-stage grouping as G1. Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus. (AJCC 7th ed.) Malignant C5023 Stage 0 Esophageal Squamous Cell Carcinoma AJCC v7 Esophageal Squamous Cell Carcinoma Stage 0 || Esophageal Squamous Cell Carcinoma in situ || Esophagus Squamous Cell Carcinoma in situ || Oesophageal Squamous Cell Carcinoma Stage 0 || Squamous Cell Carcinoma in situ of Esophagus || Squamous Cell Carcinoma in situ of the Esophagus || Stage 0 Esophageal Squamous Cell Carcinoma || Stage 0 Esophagus Squamous Cell Carcinoma || Stage 0 Squamous Cell Carcinoma of Esophagus || Stage 0 Squamous Cell Carcinoma of the Esophagus Stage 0 includes: Tis (HGD), N0, M0, G1, GX, Tumor location: Any. Tis: High-grade dysplasia. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well differentiated. GX: Grade cannot be assessed-stage grouping as G1. Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus. (AJCC 7th ed.) Malignant C4439 Stage 0 Gallbladder Cancer AJCC v6 and v7 Carcinoma in situ of Gallbladder || Carcinoma in situ of the Gallbladder || Gallbladder Cancer Stage 0 || Gallbladder Carcinoma in situ || Stage 0 Carcinoma of Gallbladder || Stage 0 Carcinoma of the Gallbladder || Stage 0 Gallbladder Cancer AJCC v6 || Stage 0 Gallbladder Cancer AJCC v7 || Stage 0 Gallbladder Carcinoma Stage 0 includes: (Tis, N0, M0). Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th Eds.) Malignant C7788 Stage 0 Gastric Cancer AJCC v6 and v7 Carcinoma in situ of Stomach || Carcinoma in situ of the Stomach || Carcinoma of Stomach Stage 0 || Carcinoma of the Stomach Stage 0 || Gastric Carcinoma Stage 0 || Gastric Carcinoma in situ || Gastric Carcinoma, Stage 0 || Stage 0 Carcinoma of Stomach || Stage 0 Carcinoma of the Stomach || Stage 0 Gastric Cancer || Stage 0 Gastric Cancer AJCC v6 || Stage 0 Gastric Cancer AJCC v7 || Stage 0 Gastric Carcinoma || Stage 0 Stomach Cancer || Stage 0 Stomach Carcinoma || Stomach Carcinoma Stage 0 || Stomach Carcinoma in situ Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial tumor without invasion of the lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C27467 Stage 0 Lung Cancer AJCC v6 and v7 Bronchial Carcinoma in situ || Stage 0 Lung Cancer AJCC v6 || Stage 0 Lung Cancer AJCC v7 || Stage 0 Lung Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C5729 Stage 0 Pancreatic Cancer AJCC v6 and v7 Pancreatic Cancer Stage 0 || Pancreatic Carcinoma in Situ || Stage 0 Carcinoma of Pancreas || Stage 0 Carcinoma of the Pancreas || Stage 0 Exocrine and Endocrine Pancreatic Cancer AJCC v6 and v7 || Stage 0 Pancreatic Cancer AJCC v6 || Stage 0 Pancreatic Cancer AJCC v7 || Stage 0 Pancreatic Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th Eds.) Malignant C4853 Stage 0 Rectal Cancer AJCC v6 and v7 Carcinoma in situ of Rectum || Carcinoma in situ of the Rectum || Marked Dysplasia of Rectum || Marked Dysplasia of the Rectum || Marked Rectal Dysplasia || Rectal Carcinoma in situ || Severe Dysplasia of Rectum || Severe Dysplasia of the Rectum || Severe Rectal Dysplasia || Stage 0 Carcinoma of Rectum || Stage 0 Carcinoma of the Rectum || Stage 0 Rectal Cancer AJCC v6 || Stage 0 Rectal Cancer AJCC v7 || Stage 0 Rectal Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial or invasion of the lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C5017 Stage 0 Rectosigmoid Cancer AJCC v6 and v7 Stage 0 Rectosigmoid Cancer AJCC v6 || Stage 0 Rectosigmoid Cancer AJCC v7 || Stage 0 Rectosigmoid Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ: intraepithelial or invasion of the lamina propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C6193 Stage 0 Renal Pelvis Cancer AJCC v7 Stage 0 Carcinoma of Renal Pelvis || Stage 0 Carcinoma of the Renal Pelvis || Stage 0 Renal Pelvis Urothelial Carcinoma || Stage 0 Renal Pelvis Urothelial Carcinoma AJCC v7 Stage 0 includes: 0a (Ta, N0, M0); 0is (Tis, N0, M0). Ta: Papillary noninvasive carcinoma. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C4892 Stage 0 Small Intestinal Cancer AJCC v7 Small Intestine Cancer Stage 0 || Small Intestine Carcinoma in situ || Stage 0 Small Intestinal Cancer || Stage 0 Small Intestine Carcinoma Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C27093 Stage 0 Squamous Cell Carcinoma Epidermoid Carcinoma in situ || Epidermoid Cell Carcinoma in situ || Grade 3 Squamous Intraepithelial Neoplasia || Grade III Squamous Intraepithelial Neoplasia || Intraepithelial Squamous Cell Carcinoma || Squamous Carcinoma in situ || Squamous Cell Carcinoma in situ || Squamous Cell Carcinoma in-situ A malignant epithelial neoplasm confined to the squamous epithelium, without invasion of the underlying tissues. Malignant C4116 Stage 0 Transitional Cell Carcinoma Transitional Carcinoma in situ || Transitional Cell Carcinoma in situ A lesion in which the surface epithelium of the bladder or the renal pelvis and ureter contains transitional cells which display malignant cytologic characteristics. There is no evidence of stromal invasion. Malignant C6149 Stage 0 Ureter Cancer AJCC v7 Stage 0 Carcinoma of Ureter || Stage 0 Carcinoma of the Ureter || Stage 0 Ureter Carcinoma || Stage 0 Ureter Urothelial Carcinoma || Stage 0 Ureter Urothelial Carcinoma AJCC v7 || Stage 0 Ureteral Carcinoma || Ureter Cancer Stage 0 Stage 0 includes: 0a (Ta, N0, M0); 0is (Tis, N0, M0). Ta: Papillary noninvasive carcinoma. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C9267 Stage 0a Bladder Urothelial Carcinoma AJCC v6 and v7 Non-Invasive Papillary Carcinoma of Bladder || Non-Invasive Papillary Carcinoma of Urinary Bladder || Non-Invasive Papillary Carcinoma of the Bladder || Non-Invasive Papillary Carcinoma of the Urinary Bladder || Non-Invasive Papillary Urothelial Carcinoma || Stage 0a Bladder Cancer || Stage 0a Bladder Carcinoma || Stage 0a Bladder Urothelial Carcinoma || Stage 0a Bladder Urothelial Carcinoma AJCC v6 || Stage 0a Bladder Urothelial Carcinoma AJCC v7 || Stage 0a Carcinoma of Bladder || Stage 0a Carcinoma of Urinary Bladder || Stage 0a Carcinoma of the Bladder || Stage 0a Carcinoma of the Urinary Bladder || Stage 0a Urinary Bladder Carcinoma Stage 0a includes: Ta, N0, M0. Ta: Papillary noninvasive carcinoma. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C6202 Stage 0a Renal Pelvis Cancer AJCC v7 Renal Pelvis Papillary Noninvasive Carcinoma || Stage 0a Carcinoma of Renal Pelvis || Stage 0a Carcinoma of the Renal Pelvis || Stage 0a Renal Pelvis Urothelial Carcinoma || Stage 0a Renal Pelvis Urothelial Carcinoma AJCC v7 Stage 0a includes: Ta, N0, M0. Ta: Papillary noninvasive carcinoma. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C3644 Stage 0is Bladder Urothelial Carcinoma AJCC v6 and v7 Bladder Flat CIS || Bladder Flat Carcinoma in Situ || Carcinoma in situ of Urinary Bladder || Carcinoma in situ of the Urinary Bladder || Flat CIS of the Bladder || Flat CIS of the Urinary Bladder || Flat Carcinoma in Situ of the Bladder || Flat Carcinoma in Situ of the Urinary Bladder || High Grade Bladder Intraurothelial Neoplasia || Stage 0is Bladder Cancer || Stage 0is Bladder Carcinoma || Stage 0is Bladder Urothelial Carcinoma || Stage 0is Bladder Urothelial Carcinoma AJCC v6 || Stage 0is Bladder Urothelial Carcinoma AJCC v7 || Stage 0is Carcinoma of Bladder || Stage 0is Carcinoma of Urinary Bladder || Stage 0is Carcinoma of the Bladder || Stage 0is Carcinoma of the Urinary Bladder || Stage 0is Urinary Bladder Carcinoma || Urinary Bladder Flat CIS || Urinary Bladder Flat Carcinoma in Situ Stage 0is includes: Tis, N0, M0. Tis: Carcinoma in situ: "flat tumor". N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.) Malignant C4597 Stage 0is Renal Pelvis Cancer AJCC v7 Carcinoma in situ of Kidney Pelvis || Carcinoma in situ of Renal Pelvis || Carcinoma in situ of the Kidney Pelvis || Carcinoma in situ of the Renal Pelvis || Kidney Pelvis Carcinoma in situ || Renal Pelvis Carcinoma in situ || Stage 0is Carcinoma of Kidney Pelvis || Stage 0is Carcinoma of Renal Pelvis || Stage 0is Carcinoma of the Kidney Pelvis || Stage 0is Carcinoma of the Renal Pelvis || Stage 0is Kidney Pelvis Carcinoma || Stage 0is Renal Pelvis Urothelial Carcinoma || Stage 0is Renal Pelvis Urothelial Carcinoma AJCC v7 Stage 0is includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 7th ed.) Malignant C9148 Stromal Predominant Kidney Wilms Tumor Stromal Predominant Kidney Adenosarcoma || Stromal Predominant Nephroblastoma || Stromal Predominant Renal Adenosarcoma || Stromal Predominant Renal Wilm's Tumor || Stromal Predominant Renal Wilms Tumor || Stromal Predominant Renal Wilms' Tumor Wilms tumor of the kidney characterized by the predominance of the mesenchymal component. Malignant C6918 Subcutaneous Panniculitis-Like T-Cell Lymphoma Subcutaneous Panniculitis-Like T-Cell Lymphoma (Alpha/Beta Type) || Subcutaneous Panniculitis-Like T-Cell Lymphoma, Alpha/Beta Type A cytotoxic primary cutaneous T-cell lymphoma. Recent studies suggest there are at least two groups of subcutaneous panniculitis-like T-cell lymphomas, each with distinct histologic features, immunophenotypic profile, and prognosis. One group has an alpha/beta, CD8 positive phenotype, involves only subcutaneous tissues, and usually has an indolent clinical course. The second group has a gamma/delta phenotype, is CD8 negative, often co-expresses CD56, is not confined to the subcutaneous tissues, and usually has a poor prognosis. In the recent WHO-EORTC classification, the term subcutaneous panniculitis-like T-cell lymphoma is reserved for cases with an alpha/beta, CD8 positive phenotype. Cases with a gamma/delta phenotype are included in the group of cutaneous gamma/delta T-cell lymphomas. Malignant C3696 Subependymal Giant Cell Astrocytoma SEGA || Subependymal Giant Cell Astrocytic Neoplasm || Subependymal Giant Cell Astrocytic Tumor A benign, slowly growing tumor (WHO grade I) typically arising in the wall of the lateral ventricles and composed of large ganglioid astrocytes. It is the most common CNS neoplasm in patients with tuberous sclerosis complex and typically occurs during the first two decades of life. (WHO) Undetermined C3795 Subependymoma Subependymal Astrocytoma || Subependymal Glioma || WHO Grade I Ependymal Neoplasm || WHO Grade I Ependymal Tumor A benign, slow growing neoplasm which is typically attached to a ventricular wall. It is composed of glial tumor cell clusters embedded in an abundant fibrillary matrix with frequent microcystic change. Some lesions have the histological features of both subependymoma and ependymoma. It is often detected incidentally and has a very favorable prognosis. (Adapted from WHO.) Undetermined C8187 Subglottic Squamous Cell Carcinoma Epidermoid Carcinoma of Subglottis || Epidermoid Carcinoma of the Subglottis || Squamous Cell Carcinoma of Subglottis || Squamous Cell Carcinoma of the Subglottis || Subglottic Epidermoid Carcinoma || Subglottis Epidermoid Carcinoma || Subglottis Squamous Cell Carcinoma A squamous cell carcinoma of the larynx that arises from the subglottic area. Symptoms include dyspnea and stridor. It spreads to the hypopharynx, trachea, and thyroid gland. Malignant C5958 Sublingual Gland Adenocarcinoma Adenocarcinoma of Sublingual Gland || Adenocarcinoma of the Sublingual Gland An adenocarcinoma that arises from the sublingual gland. Malignant C8397 Sublingual Gland Carcinoma Carcinoma of Sublingual Gland || Carcinoma of the Sublingual Gland A carcinoma that arises from the sublingual gland. Representative examples include cystadenocarcinoma and mucoepidermoid carcinoma. Malignant C5940 Submandibular Gland Adenocarcinoma Adenocarcinoma of Submandibular Gland || Adenocarcinoma of the Submandibular Gland An adenocarcinoma that arises from the submandibular gland. Representative examples include polymorphic low-grade adenocarcinoma and acinic cell carcinoma. Malignant C5935 Submandibular Gland Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Submandibular Gland || Adenoid Cystic Carcinoma of the Submandibular Gland An aggressive carcinoma that arises from the submandibular gland. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Patients develop pain because of the tendency of these carcinomas to invade perineural tissues. Malignant C8396 Submandibular Gland Carcinoma Carcinoma of Submandibular Gland || Carcinoma of the Submandibular Gland A carcinoma that arises from the submandibular gland. Representative examples include mucoepidermoid carcinoma, adenoid cystic carcinoma, and carcinoma ex pleomorphic adenoma. Malignant C5977 Submandibular Gland Carcinoma ex Pleomorphic Adenoma Submaxillary Gland Carcinoma ex Pleomorphic Adenoma A carcinoma that arises from a pleomorphic adenoma in the submandibular gland. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive. Malignant C5939 Submandibular Gland Mucoepidermoid Carcinoma Mucoepidermoid Carcinoma of Submandibular Gland || Mucoepidermoid Carcinoma of the Submandibular Gland A carcinoma that arises from the submandibular gland. It usually presents as a firm and painless mass. It is characterized by the presence of epidermoid cells, mucus producing cells, and cells of intermediate type. The majority of cases have a favorable outcome. Malignant C5943 Submandibular Gland Squamous Cell Carcinoma Squamous Cell Carcinoma of Submandibular Gland || Squamous Cell Carcinoma of the Submandibular Gland An invasive squamous cell carcinoma that arises from the submandibular gland. It usually affects elderly patients and presents as a rapidly enlarging tumor mass, often associated with pain. Malignant C5947 Submandibular Gland Undifferentiated Carcinoma Anaplastic Carcinoma of Submandibular Gland || Anaplastic Carcinoma of the Submandibular Gland || Undifferentiated Carcinoma of Submandibular Gland || Undifferentiated Carcinoma of the Submandibular Gland A carcinoma that arises from the submandibular gland and is characterized by the presence of undifferentiated, anaplastic malignant epithelial cells. Malignant C6814 Superficial Fibromatosis A poorly circumscribed, intermediate fibrocytic neoplasm arising from the superficial soft tissues. It is characterized by the presence of spindle-shaped fibroblasts, and an infiltrative growth pattern. Undetermined C4945 Supraglottic Squamous Cell Carcinoma Epidermoid Carcinoma of Supraglottis || Epidermoid Carcinoma of the Supraglottis || Squamous Cell Carcinoma of Supraglottis || Squamous Cell Carcinoma of the Supraglottis || Supraglottic Epidermoid Carcinoma || Supraglottis Epidermoid Carcinoma || Supraglottis Squamous Cell Carcinoma A squamous cell carcinoma of the larynx that arises from the supraglottic area. Signs and symptoms include dysphagia, a sensation of foreign body in the throat, and hemoptysis. It spreads to the space anterior to the epiglottis, pyriform sinus, and base of the tongue. Malignant C6968 Supratentorial Embryonal Tumor, Not Otherwise Specified Supratentorial Embryonal Tumor, NOS A central nervous system embryonal tumor, not otherwise specified arising from the supratentorial region. Malignant C6938 Sweat Gland Carcinoma Carcinoma of Sweat Gland || Carcinoma of the Sweat Gland A carcinoma arising from the sweat glands. Representative examples include tubular carcinoma, spiradenocarcinoma, eccrine carcinoma, hidradenocarcinoma, and apocrine carcinoma. Malignant C3761 Syringoma Eccrine Syringoma A benign sweat gland neoplasm usually affecting the lower eyelids and upper cheeks. The lesions are papular and are usually numerous. Morphologically, there are nests, cords, and tubules of epithelial cells present, surrounded by a dense stroma in the reticular dermis. Benign C9235 Systemic Mastocytosis Systemic Tissue Mast Cell Disease A variant of mastocytosis characterized by multifocal, dense infiltrates of mast cells (15 or more mast cells in aggregates) detected in the bone marrow and/or other extracutaneous sites. (WHO, 2001) Malignant C9284 Systemic Mastocytosis with an Associated Hematological Neoplasm Systemic Mastocytosis with Associated Clonal Hematological non-Mast-Cell Lineage Disease || Systemic Mastocytosis with an Associated Hematological Neoplasm (SM-AHN) A disorder characterized by systemic infiltration of internal organs by aggregates of neoplastic mast cells and the presence of a clonal non-mast cell hematologic neoplasm (e.g., myelodysplastic syndrome, chronic myeloproliferative disorder, acute myeloid leukemia, and lymphoma). Malignant C3183 T Acute Lymphoblastic Leukemia Acute T Cell Leukemia || Acute T Cell Lymphoblastic Leukemia || Acute T-Cell Leukemia || Acute T-Cell Lymphoblastic Leukemia || Precursor T-Lymphoblastic Leukemia || T-Cell Acute Lymphoblastic Leukemia || T-Cell Type Acute Leukemia Acute lymphoblastic leukemia of T-cell origin. It comprises about 15% of childhood cases and 25% of adult cases. It is more common in males than females. (WHO, 2001) Malignant C8694 T Lymphoblastic Leukemia/Lymphoma Precursor T Lymphoblastic Leukemia/Lymphoma || Precursor T Lymphoblastic Lymphoma/Leukemia || Precursor T-Lymphoblastic Lymphoma/Leukemia A neoplasm of lymphoblasts committed to the T-cell lineage, typically composed of small to medium-sized blast cells. When the neoplasm involves predominantly the bone marrow and the peripheral blood, it is called T acute lymphoblastic leukemia. When it involves nodal or extranodal sites it is called T lymphoblastic lymphoma. (WHO, 2001) Malignant C6919 T Lymphoblastic Lymphoma Precursor T Lymphoblastic Lymphoma || Precursor T-Cell Lymphoblastic Lymphoma || Precursor T-Lymphoblastic Lymphoma || T-Lymphoblastic Lymphoma The most frequent type of lymphoblastic lymphoma. It comprises approximately 85-90% of cases. It is more frequently seen in adolescent males. It frequently presents with a mass lesion in the mediastinum. Pleural effusions are common. (WHO, 2001) Malignant C6903 Tanycytic Ependymoma A variant of ependymoma, often found in the spinal cord, with tumor cells arranged in fascicles of variable width and cell density. Ependymal rosettes are generally absent, so this lesion must be distinguished from astrocytic neoplasms, but its EM characteristics are ependymal. (Adapted from WHO.) Undetermined C4664 T-Cell Large Granular Lymphocyte Leukemia Large Cell Granular Lymphoid Leukemia || Large Granular Lymphocytic Leukemia || Large Granular Lymphocytosis || T Gamma Lymphoproliferative Disorder || T-Cell Large Granular Lymphocytic Leukemia || T-Gamma Lymphoproliferative Disorder A T-cell peripheral neoplasm characterized by a persistent (>6 months) increase in the number of peripheral blood large granular lymphocytes, without a clearly identified cause. (WHO, 2001) Malignant C39586 T-Cell Large Granular Lymphocyte Leukemia Expressing the T-Cell Receptor Gamma-Delta T-Cell Large Granular Lymphocyte Leukemia Expressing the TCR Gamma-Delta An uncommon variant of T-cell large granular lymphocyte leukemia expressing the gamma-delta T-cell receptor. Approximately 60% of these cases are CD8-positive and the remainder are CD4/CD8-negative. Malignant C39584 T-Cell Large Granular Lymphocyte Leukemia, Common Variant T-cell large granular lymphocyte leukemia defined by the presence of CD3-positive, CD8-positive, and T-cell receptor alpha-beta-positive cytotoxic T-cells. Malignant C3466 T-Cell Non-Hodgkin Lymphoma Non-Hodgkin's T-Cell Lymphoma || T Cell Lymphoma || T-Cell Lymphoma || T-Cell Non-Hodgkin's Lymphoma || T-Cell and NK-Cell Non-Hodgkin Lymphoma || T-Cell and NK-Cell Non-Hodgkin's Lymphoma A non-Hodgkin lymphoma of T-cell lineage. It includes the T lymphoblastic lymphoma and the mature T- and NK-cell lymphomas. Malignant C27897 T-Cell Proliferation of Uncertain Malignant Potential A T-cell lymphoproliferative disorder with uncertain malignant potential. A representative example is lymphomatoid papulosis. Uncertain Malignant Potential C4752 T-Cell Prolymphocytic Leukemia T Cell Prolymphocytic Leukemia || T Prolymphocytic Leukemia An aggressive T-cell leukemia, characterized by the proliferation of small to medium sized prolymphocytes with a mature T-cell phenotype, involving the blood, bone marrow, lymph nodes, liver, spleen, and skin. (WHO, 2001) Malignant C36271 T-Cell Prolymphocytic Leukemia, Cerebriform Cell Variant T-Cell Prolymphocytic Leukemia, Sezary Cell-like Variant || T-Cell Prolymphocytic Leukemia, Sézary Cell-like Variant A T-cell prolymphocytic leukemia characterized by the presence of neoplastic prolymphocytes with irregular or cerebriform nuclear outline. Malignant C36270 T-Cell Prolymphocytic Leukemia, Small Cell Variant A T-cell prolymphocytic leukemia characterized by the presence of small neoplastic prolymphocytes often with invisible nucleolus by light microscopy. Malignant C9496 T-Cell/Histiocyte-Rich Large B-Cell Lymphoma T-Cell Rich/Histiocyte-Rich Large B-Cell Lymphoma || T-Cell/Histiocyte Rich Lymphoma || THRLBCL A large B-cell lymphoma characterized by the presence of a limited number of scattered neoplastic large B-lymphocytes which are admixed with numerous non-neoplastic T-lymphocytes and frequently histiocytes. Malignant C3402 Tenosynovial Giant Cell Tumor Fibrous Histiocytoma of Tendon Sheath || Giant Cell Neoplasm of Tendon Sheath || Giant Cell Neoplasm of Tenosynovium || Giant Cell Neoplasm of the Tenosynovium || Giant Cell Tumor of Tendon Sheath || Giant Cell Tumor of Tenosynovium || Giant Cell Tumor of the Tenosynovium || Tendon Sheath Giant Cell Neoplasm || Tendon Sheath Giant Cell Tumor || Tenosynovial Giant Cell Neoplasm A tumor usually arising in the synovium of joints, bursa or tendon sheath. It is characterized by the presence of mononuclear cells, multinucleated osteoclast-like giant cells, hemosiderin-laden macrophages, foam cells, and an inflammatory infiltrate. According to the growth pattern, it is classified as localized or diffuse. Undetermined C3403 Teratoma A non-seminomatous germ cell tumor characterized by the presence of various tissues which correspond to the different germinal layers (endoderm, mesoderm, and ectoderm). It occurs in the testis, ovary, and extragonadal sites including central nervous system, mediastinum, lung, and stomach. According to the level of differentiation of the tissues which comprise the tumor, teratomas are classified as benign (grade 0 or 1), immature (grade 2), and malignant (grade 3). Grade 0 teratomas contain only mature elements; grade 1 teratomas have a limited degree of immaturity; grade 2 teratomas have a more extensive degree of immaturity; grade 3 teratomas are composed exclusively of immature tissues. The prognosis depends on patient age, tumor size and grade, and stage. Undetermined C7733 Testicular Choriocarcinoma Choriocarcinoma of Testis || Choriocarcinoma of the Testis A malignant germ cell tumor arising from the testis. It represents the rarest of the testicular germ cell tumors. Histologically it is characterized by the presence of syncytiotrophoblasts and cytotrophoblasts. Malignant C8591 Testicular Germ Cell Tumor Germ Cell Neoplasm of Testis || Germ Cell Neoplasm of the Testis || Germ Cell Tumor of Testis || Germ Cell Tumor of the Testis || Testicular Germ Cell Neoplasm || Testicular Germ Cell Neoplasms A germ cell tumor arising from the testis. Representative examples include teratoma, seminoma, embryonal carcinoma, and yolk sac tumor. Undetermined C7328 Testicular Seminoma Seminoma of Testis || Seminoma of the Testis || Testicular Seminoma Pure A malignant germ cell tumor arising from the testis. It is believed that it is derived from the sexually undifferentiated embryonic gonad. Treatment with radiotherapy is highly successful when the tumor is diagnosed in localized stages, which represents the majority of presentations of seminoma. Malignant C80683 Therapy-Related Leukemia Therapy Related Leukemia A leukemia arising as a result of the mutagenic effect of chemotherapy agents that are used for the treatment of a malignant tumor or exposure to ionizing radiation. Malignant C27722 Therapy-Related Myelodysplastic Syndrome Therapy Related Myelodysplastic Syndrome || Therapy-Related MDS A myelodysplastic syndrome caused by chemotherapy and/or radiotherapy. Malignant C82397 Therapy-Related Myelodysplastic/Myeloproliferative Neoplasm Therapy-Related Myelodysplastic/Myeloproliferative Disease A myelodysplastic/myeloproliferative neoplasm arising as a result of the mutagenic effect of chemotherapy agents and/or radiation that are used for the treatment of neoplastic or non-neoplastic disorders. Malignant C6459 Thymic Adenocarcinoma Adenocarcinoma of Thymus || Adenocarcinoma of the Thymus || Thymus Adenocarcinoma A rare primary thymic carcinoma, characterized by the presence of carcinoma cells with glandular differentiation. Malignant C6458 Thymic Adenosquamous Carcinoma Adenosquamous Carcinoma of Thymus || Adenosquamous Carcinoma of the Thymus || Thymus Adenosquamous Carcinoma A rare carcinoma that arises from the thymus and is characterized by the presence of glandular and squamous carcinomatous components. Malignant C6430 Thymic Carcinoid Tumor Carcinoid Tumor of Thymus || Carcinoid Tumor of the Thymus || Thymic Carcinoid || Thymus Carcinoid || Thymus Carcinoid Tumor A primary thymic neuroendocrine tumor, often associated with Cushing syndrome. Two morphologic subtypes are recognized: typical and atypical carcinoid tumors. Atypical carcinoid tumors have a more aggressive clinical course. Undetermined C7569 Thymic Carcinoma Thymic Carcinoma (Excluding Well Differentiated Thymic Carcinoma) || Thymic Carcinoma Excluding Well Differentiated Thymic Carcinoma A diverse group of carcinomas of the thymus gland, previously known as thymoma type C. It includes morphologic variants derived from purely epithelial cells, as well as from cells with neuroendocrine differentiation. Malignant C6462 Thymic Clear Cell Carcinoma Clear Cell Carcinoma of Thymus || Clear Cell Carcinoma of the Thymus || Thymus Clear Cell Carcinoma A rare, usually aggressive, primary thymic carcinoma, characterized by the presence of carcinoma cells with clear cytoplasm. Malignant C6461 Thymic Large Cell Neuroendocrine Carcinoma Large Cell Neuroendocrine Carcinoma of Thymus || Large Cell Neuroendocrine Carcinoma of the Thymus || Thymic LCNEC A large cell neuroendocrine carcinoma arising from the thymus. Malignant C7998 Thymic Lymphoepithelioma-Like Carcinoma Lymphoepithelial Cancer of Thymus || Lymphoepithelial Cancer of the Thymus || Lymphoepithelial Thymus Cancer || Lymphoepithelioma-like Carcinoma of Thymus || Lymphoepithelioma-like Carcinoma of the Thymus || Lymphoepithelioma-like Thymic Carcinoma || Lymphoepithelioma-like Thymus Carcinoma || Malignant Lymphoepithelial Thymoma A rare, usually aggressive primary thymic carcinoma, characterized by a syncytial growth of undifferentiated carcinoma cells and the presence of a lymphoplasmacytic infiltrate. More than 40% of cases are associated with Epstein-Barr virus infection. Malignant C6457 Thymic Mucoepidermoid Carcinoma Mucoepidermoid Carcinoma of Thymus || Mucoepidermoid Carcinoma of the Thymus || Thymus Mucoepidermoid Carcinoma A rare primary thymic carcinoma, characterized by the presence of squamous cells, intermediate type cells, and mucus-producing cells. The prognosis depends on histologic grade and stage. Malignant C6463 Thymic Sarcomatoid Carcinoma Sarcomatoid Carcinoma of Thymus || Sarcomatoid Carcinoma of the Thymus || Thymic Carcinosarcoma || Thymic Spindle Cell Carcinoma || Thymus Sarcomatoid Carcinoma A rare, usually aggressive, primary thymic carcinoma, characterized by the presence of tumor cells morphologically resembling soft tissue sarcoma. Malignant C6460 Thymic Small Cell Neuroendocrine Carcinoma Small Cell Carcinoma of Thymus || Small Cell Carcinoma of the Thymus || Thymic Small Cell Carcinoma || Thymic Small Cell Carcinoma Neuroendocrine Type || Thymus Small Cell Carcinoma A small cell neuroendocrine carcinoma arising from the thymus. Malignant C6455 Thymic Squamous Cell Carcinoma Epidermoid Thymic Carcinoma || Squamous Cell Carcinoma of Thymus || Squamous Cell Carcinoma of the Thymus || Thymus Squamous Cell Carcinoma A rare primary thymic carcinoma, characterized by the presence of keratinizing or non-keratinizing malignant squamous cells. Approximately 10-20% of cases occur in combination with thymoma. The prognosis depends on the tumor stage and the degree of cellular differentiation. Malignant C35718 Thymic Undifferentiated Carcinoma A rare primary thymic carcinoma, characterized by an undifferentiated solid tumor growth, without associated sarcomatoid features. Malignant C3411 Thymoma A neoplasm arising from the epithelial cells of the thymus. Although thymomas are usually encapsulated tumors, they may invade the capsule and infiltrate the surrounding tissues or even metastasize to distant anatomic sites. The following morphologic subtypes are currently recognized: type A, type B, type AB, metaplastic, micronodular, microscopic, and sclerosing thymoma. Thymomas type B are further subdivided into types B1, B2, and B3. Thymoma type B3 usually has the most aggressive clinical course. Undetermined C6454 Thymoma Type A Medullary Thymoma || Spindle Cell Thymoma A thymic epithelial neoplasm characterized by the presence of spindle and/or oval neoplastic epithelial cells. Lymphocytic infiltration is minimal or absent. It may be associated with myasthenia gravis or pure red cell aplasia. The majority of cases occur in the anterior mediastinum as Masaoka stage I tumors. Approximately 20% of the cases occur as stage II or stage III tumors. Type A thymoma generally behaves as a benign tumor and the overall survival is reported to be 100% at 5 and 10 years. Undetermined C6885 Thymoma Type AB Mixed Type Thymoma A thymic epithelial neoplasm characterized by the presence of a lymphocyte-poor component similar to that seen in type A thymoma and a lymphocyte-rich component which contains neoplastic small polygonal epithelial cells. It may be associated with myasthenia gravis and pure red cell aplasia. The majority of cases occur in the anterior mediastinum as Masaoka stage I tumors. A minority of the cases occur as stage II or stage III tumors. The overall survival is reported to be 80-100% at 5 and 10 years. Undetermined C7114 Thymoma Type B Dendritic Cell Thymoma || Epithelioid Thymoma || Plump Cell Thymoma An epithelial neoplasm arising from the thymus. It may be associated with myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. It includes thymoma type B1 which is a thymoma of low grade malignant potential, thymoma type B2 which is a thymoma of moderate malignancy, and thymoma type B3 which is also known as well differentiated thymic carcinoma. Undetermined C6887 Thymoma Type B1 Lymphocyte-Predominant Thymoma || Lymphocyte-Rich Thymoma || Organoid Thymoma || Predominantly Cortical Thymoma A thymic epithelial neoplasm characterized by the presence of expanded areas which resemble the normal thymic cortex. The neoplastic epithelial cells are small and scant and there is a dense T-lymphocytic component present. Areas of medullary differentiation with or without Hassall's corpuscles are also present. It may be associated with myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. It has a low grade malignant potential. The majority of cases occur in the anterior mediastinum as Masaoka stage I tumors. A minority of the cases occur as stage II tumors. Undetermined C6888 Thymoma Type B2 Cortical Thymoma || Polygonal Cell Thymoma A thymic epithelial neoplasm characterized by the presence of neoplastic large, polygonal epithelial cells with large vesicular nuclei and prominent nucleoli. The neoplastic cells are arranged around perivascular spaces and along septa. Immature T-lymphocytes are also present. It may be associated with myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. It is a tumor of moderate malignancy. The majority of cases occur in the anterior mediastinum as Masaoka stage I, stage II, or stage III tumors. Metastatic, stage IV tumors occur less frequently. Undetermined C7997 Thymoma Type B3 Atypical Thymoma || Epithelial Malignant Thymoma || Epithelial Thymoma || Malignant Thymoma Type B3 || Squamoid Thymoma || Well Differentiated Thymic Carcinoma || Well-Differentiated Thymic Carcinoma Also known as well-differentiated thymic carcinoma, atypical thymoma, or epithelial thymoma, this type of thymoma displays morphologic characteristics of a well-differentiated carcinoma. The majority of cases occur in the anterior mediastinum as Masaoka stage II or stage III tumors. It is almost always invasive, it recurs frequently, and metastasizes in approximately 20% of the cases. Malignant C27380 Thyroid Gland Adenocarcinoma Thyroid Adenocarcinoma An adenocarcinoma arising from the follicular cells of the thyroid gland. According to the degree of differentiation, it is classified either as differentiated carcinoma (extensive evidence of follicular cell differentiation), or poorly differentiated carcinoma (limited evidence of follicular cell differentiation). Malignant C3878 Thyroid Gland Anaplastic Carcinoma Anaplastic Carcinoma of Thyroid || Anaplastic Carcinoma of Thyroid Gland || Anaplastic Carcinoma of the Thyroid || Anaplastic Carcinoma of the Thyroid Gland || Anaplastic Thyroid Cancer || Anaplastic Thyroid Carcinoma || Anaplastic Thyroid Gland Carcinoma || Dedifferentiated Thyroid Gland Carcinoma || Metaplastic Thyroid Gland Carcinoma || Pleomorphic Thyroid Gland Carcinoma || Sarcomatoid Thyroid Gland Carcinoma || Thyroid Gland Carcinosarcoma || Thyroid Gland Giant Cell Carcinoma || Thyroid Gland Metaplastic Carcinoma || Thyroid Gland Pleomorphic Carcinoma || Thyroid Gland Sarcomatoid Carcinoma || Thyroid Gland Spindle Cell Carcinoma || Thyroid Gland Undifferentiated (Anaplastic) Carcinoma || Undifferentiated (Anaplastic) Thyroid Gland Carcinoma || Undifferentiated Carcinoma of Thyroid || Undifferentiated Carcinoma of Thyroid Gland || Undifferentiated Carcinoma of the Thyroid || Undifferentiated Carcinoma of the Thyroid Gland || Undifferentiated Thyroid Carcinoma || Undifferentiated Thyroid Gland Carcinoma A primary carcinoma of the thyroid gland composed of undifferentiated cells. The malignant cells demonstrate evidence of epithelial differentiation, either by immunohistochemistry or electron microscopic studies. Microscopically, in the majority of cases there is a mixture of spindle, epithelioid, and giant cells. The vast majority of the patients present with a rapidly enlarging neck mass. The clinical course is usually aggressive. Malignant C4815 Thyroid Gland Carcinoma Cancer of Thyroid || Cancer of the Thyroid || Carcinoma of Thyroid || Carcinoma of Thyroid Gland || Carcinoma of the Thyroid || Carcinoma of the Thyroid Gland || Thyroid Cancer || Thyroid Carcinoma || Thyroid Gland Cancer A carcinoma arising from the thyroid gland. It includes the following main subtypes: follicular, papillary, medullary, poorly differentiated, and undifferentiated (anaplastic) carcinoma. Malignant C3502 Thyroid Gland Follicular Adenoma Adenoma of Thyroid || Adenoma of Thyroid Gland || Adenoma of the Thyroid || Adenoma of the Thyroid Gland || Follicular Adenoma || Follicular Adenoma of Thyroid || Follicular Adenoma of Thyroid Gland || Follicular Adenoma of the Thyroid || Follicular Adenoma of the Thyroid Gland || Thyroid Adenoma || Thyroid Follicular Adenoma || Thyroid Gland Adenoma A benign, encapsulated tumor, arising from the follicular cells of the thyroid gland. It may be associated with thyroid hormone secretion but it does not have malignant characteristics. Benign C8054 Thyroid Gland Follicular Carcinoma Follicular Adenocarcinoma || Follicular Cancer of Thyroid || Follicular Cancer of Thyroid Gland || Follicular Cancer of the Thyroid || Follicular Cancer of the Thyroid Gland || Follicular Carcinoma || Follicular Carcinoma of Thyroid || Follicular Carcinoma of Thyroid Gland || Follicular Carcinoma of the Thyroid || Follicular Carcinoma of the Thyroid Gland || Follicular Thyroid Cancer || Follicular Thyroid Carcinoma || Follicular Thyroid Gland Carcinoma || Thyroid Follicular Carcinoma || Well-Differentiated Follicular Adenocarcinoma || Well-Differentiated Follicular Carcinoma A differentiated adenocarcinoma arising from the follicular cells of the thyroid gland. The nuclear features which characterize the thyroid gland papillary carcinoma are absent. Radiation exposure is a risk factor and it comprises approximately 10% to 15% of thyroid cancers. Clinically, it usually presents as a solitary mass in the thyroid gland. It is generally unifocal and thickly encapsulated and shows invasion of the capsule or the vessels. Diagnostic procedures include thyroid ultrasound and fine needle biopsy. Malignant C3879 Thyroid Gland Medullary Carcinoma C Cell Carcinoma || C-Cell Carcinoma || Medullary Carcinoma || Medullary Carcinoma of Thyroid || Medullary Carcinoma of Thyroid Gland || Medullary Carcinoma of the Thyroid || Medullary Carcinoma of the Thyroid Gland || Medullary Thyroid Cancer || Medullary Thyroid Carcinoma || Medullary Thyroid Gland Carcinoma || Parafollicular Cell Carcinoma || Thyroid Gland Neuroendocrine Carcinoma || Thyroid Medullary Carcinoma A neuroendocrine carcinoma arising from the C-cells of the thyroid gland. It is closely associated with multiple endocrine neoplasia syndromes. Approximately 10% to 20% of medullary thyroid carcinomas are familial. Patients usually present with a thyroid nodule that is painless and firm. In the majority of cases nodal involvement is present at diagnosis. Surgery is the preferred treatment for both primary lesions and recurrences. This carcinoma is generally not very sensitive to radiation and almost unresponsive to chemotherapy. Malignant C46104 Thyroid Gland Mixed Medullary and Follicular Cell Carcinoma Thyroid Gland Mixed Medullary and Papillary Carcinoma A primary carcinoma of the thyroid gland containing a medullary carcinoma component that is immunohistochemically positive for calcitonin, and follicular cell carcinoma structures that are immunohistochemically positive for thyroglobulin. Malignant C4035 Thyroid Gland Papillary Carcinoma Papillary Cancer of Thyroid || Papillary Cancer of Thyroid Gland || Papillary Cancer of the Thyroid || Papillary Cancer of the Thyroid Gland || Papillary Carcinoma of Thyroid || Papillary Carcinoma of Thyroid Gland || Papillary Carcinoma of the Thyroid || Papillary Carcinoma of the Thyroid Gland || Papillary Thyroid Cancer || Papillary Thyroid Carcinoma || Papillary Thyroid Gland Carcinoma || Thyroid Papillary Carcinoma A differentiated adenocarcinoma arising from the follicular cells of the thyroid gland. Radiation exposure is a risk factor and it is the most common malignant thyroid lesion, comprising 75% to 80% of all thyroid cancers in iodine sufficient countries. Diagnostic procedures include thyroid ultrasound and fine needle biopsy. Microscopically, the diagnosis is based on the distinct characteristics of the malignant cells, which include enlargement, oval shape, elongation, and overlapping of the nuclei. The nuclei also display clearing or have a ground glass appearance. Malignant C46008 Thyroid Gland Squamous Cell Carcinoma Squamous Cell Thyroid Gland Carcinoma A rapidly growing primary carcinoma of the thyroid gland composed of malignant squamous cells. The clinical course is usually aggressive. Malignant C8011 Thyrotroph Adenoma TSH Producing Adenoma of Pituitary || TSH Producing Adenoma of Pituitary Gland || TSH Producing Adenoma of the Pituitary || TSH Producing Adenoma of the Pituitary Gland || TSH Producing Pituitary Adenoma || TSH Producing Pituitary Gland Adenoma || TSH Secreting Adenoma of Pituitary || TSH Secreting Adenoma of Pituitary Gland || TSH Secreting Adenoma of the Pituitary || TSH Secreting Adenoma of the Pituitary Gland || TSH Secreting Pituitary Adenoma || TSH Secreting Pituitary Gland Adenoma || TSH-Producing Pituitary Gland Adenoma || TSHoma || Thyroid Stimulating Hormone Producing Adenoma of Pituitary || Thyroid Stimulating Hormone Producing Adenoma of Pituitary Gland || Thyroid Stimulating Hormone Producing Adenoma of the Pituitary || Thyroid Stimulating Hormone Producing Adenoma of the Pituitary Gland || Thyroid Stimulating Hormone Producing Pituitary Adenoma || Thyroid Stimulating Hormone Producing Pituitary Gland Adenoma || Thyroid Stimulating Hormone Secreting Adenoma of Pituitary || Thyroid Stimulating Hormone Secreting Adenoma of Pituitary Gland || Thyroid Stimulating Hormone Secreting Adenoma of the Pituitary || Thyroid Stimulating Hormone Secreting Adenoma of the Pituitary Gland || Thyroid Stimulating Hormone Secreting Pituitary Adenoma || Thyroid Stimulating Hormone Secreting Pituitary Gland Adenoma || Thyrotrope Adenoma || Thyrotropin Producing Pituitary Gland Adenoma A rare adenoma of the anterior lobe of the pituitary gland that produces thyrotropin. It is usually associated with goiter and hyperthyroidism. Benign C8461 Tibial Adamantinoma An adamantinoma arising from the tibia. The tibia is the site which is more frequently involved by adamantinoma (80-90% of cases). Malignant C4648 Tongue Squamous Cell Carcinoma SCC of Tongue || SCC of the Tongue || Squamous Cell Carcinoma of Tongue || Squamous Cell Carcinoma of the Tongue || Tongue SCC A squamous cell carcinoma that arises from the tongue. It usually presents as a painful ulcerated or nodular lesion. The size of the tumor and the status of the lymph nodes are the most important factors that determine prognosis. Malignant C27755 Topoisomerase II Inhibitor-Related Acute Myeloid Leukemia Topoisomerase II Inhibitor Related Acute Myeloid Leukemia Acute myeloid leukemias arising as a result of the mutagenic effect of therapeutic agents targeting DNA-topoisomerase II. The vast majority of patients present without a preceding myelodysplastic phase. The acute myeloid leukemias are usually of the monoblastic or myelomonocytic type. Clonal cytogenetic abnormalities are frequently present and usually involve chromosome 11q23 (MLL gene). Malignant C6051 Tracheal Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma of Trachea || Adenoid Cystic Carcinoma of the Trachea || Trachea Adenoid Cystic Carcinoma An adenoid cystic carcinoma that arises from the trachea. It spreads to the submucosal tracheal tissue and to regional lymph nodes. Malignant C9347 Tracheal Carcinoma Cancer of Trachea || Cancer of the Trachea || Carcinoma of Trachea || Carcinoma of the Trachea || Trachea Cancer || Trachea Carcinoma Malignant epithelial neoplasm arising from the tracheal mucosa. Malignant C4448 Tracheal Squamous Cell Carcinoma Epidermoid Carcinoma of Trachea || Epidermoid Carcinoma of the Trachea || Squamous Cell Carcinoma of Trachea || Squamous Cell Carcinoma of the Trachea || Trachea Epidermoid Carcinoma || Trachea Squamous Cell Carcinoma || Tracheal Epidermoid Carcinoma A rare squamous cell carcinoma that arises from the mucosal lining of the trachea. It usually grows as an intraluminal mass and later invades extraluminal structures. The majority of patients present with hemoptysis, coughing, dyspnea, or stridor. Malignant C2930 Transitional Cell Carcinoma Transitional Carcinoma A malignant neoplasm arising from the transitional epithelium, usually affecting the urinary bladder, ureter, or renal pelvis. It may or may not have a papillary configuration. It is graded 1 to 3 or 4 according to the degree of cellular differentiation and architectural patterns. Grade 1 transitional cell carcinoma is histologically benign but it may recur. Transitional cell carcinomas may also affect the upper respiratory tract and the ovaries. Malignant C4333 Transitional Meningioma Mixed Meningioma || Transitional (Mixed) Meningioma A WHO grade I meningioma characterized by the coexistence of meningothelial cells and fibrous architectural patterns. Undetermined C43326 Trichilemmal Carcinoma Trichilemmocarcinoma A rare malignant tumor arising from the outer hair sheath and infundibulum on the face. It is considered the malignant counterpart of tricholemmoma. Complete surgical excision is required. Malignant C4113 Trichilemmoma Tricholemmoma A benign neoplasm arising from the outer hair sheath and infundibulum. It occurs in the head and neck, usually on the face. It usually presents as an exophytic wart-like lesion or a dome-shaped lesion with smooth surface. Multiple facial lesions are usually associated with Cowden disease. Benign C27132 Trichoblastoma Brooke's Tumor || Trichoepithelioma || Trichogenic Adnexal Tumor || Trichogenic Trichoblastoma A benign hair follicle neoplasm with trichoblastic differentiation. It usually presents as a solitary papular lesion It most often presents on the head and neck area, but it may develop in any anatomic site containing hair follicles. Because of its benign nature, treatment usually is not required, provided that the diagnosis has been established with certainty. Benign C7019 Trilateral Retinoblastoma Trilateral retinoblastoma refers to bilateral (or less often unilateral) retinoblastoma associated with an intracranial primitive neuroectodermal tumor in the pineal or suprasellar region. This syndrome is often associated with a increased familial incidence of retinoblastoma. (From Cancer 86(1): 135-141, 1999). Malignant C71732 Triple-Negative Breast Carcinoma Triple-Negative Breast Cancer An invasive breast carcinoma which is negative for expression of estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor 2 (HER2). Malignant C3422 Trophoblastic Tumor Trophoblastic Neoplasm || Trophoblastic Neoplasms A gestational or non-gestational neoplasm composed of neoplastic trophoblastic cells. Representative examples include hydatidiform mole and choriocarcinoma. Undetermined C5965 TSH-Producing Pituitary Gland Carcinoma Malignant Pituitary Gland Thyrotropinoma || Malignant Pituitary Thyrotropinoma || Malignant TSH Producing Neoplasm of Pituitary || Malignant TSH Producing Neoplasm of Pituitary Gland || Malignant TSH Producing Neoplasm of the Pituitary || Malignant TSH Producing Neoplasm of the Pituitary Gland || Malignant TSH Producing Pituitary Gland Neoplasm || Malignant TSH Producing Pituitary Gland Tumor || Malignant TSH Producing Pituitary Neoplasm || Malignant TSH Producing Pituitary Tumor || Malignant TSH Producing Tumor of Pituitary || Malignant TSH Producing Tumor of Pituitary Gland || Malignant TSH Producing Tumor of the Pituitary || Malignant TSH Producing Tumor of the Pituitary Gland || Malignant TSH Secreting Neoplasm of Pituitary || Malignant TSH Secreting Neoplasm of Pituitary Gland || Malignant TSH Secreting Neoplasm of the Pituitary || Malignant TSH Secreting Neoplasm of the Pituitary Gland || Malignant TSH Secreting Pituitary Gland Neoplasm || Malignant TSH Secreting Pituitary Gland Tumor || Malignant TSH Secreting Pituitary Neoplasm || Malignant TSH Secreting Pituitary Tumor || Malignant TSH Secreting Tumor of Pituitary || Malignant TSH Secreting Tumor of Pituitary Gland || Malignant TSH Secreting Tumor of the Pituitary || Malignant TSH Secreting Tumor of the Pituitary Gland || Malignant Thyroid Stimulating Hormone Producing Neoplasm of Pituitary || Malignant Thyroid Stimulating Hormone Producing Neoplasm of Pituitary Gland || Malignant Thyroid Stimulating Hormone Producing Neoplasm of the Pituitary || Malignant Thyroid Stimulating Hormone Producing Neoplasm of the Pituitary Gland || Malignant Thyroid Stimulating Hormone Producing Pituitary Gland Neoplasm || Malignant Thyroid Stimulating Hormone Producing Pituitary Gland Tumor || Malignant Thyroid Stimulating Hormone Producing Pituitary Neoplasm || Malignant Thyroid Stimulating Hormone Producing Pituitary Tumor || Malignant Thyroid Stimulating Hormone Producing Tumor || Malignant Thyroid Stimulating Hormone Producing Tumor of Pituitary || Malignant Thyroid Stimulating Hormone Producing Tumor of Pituitary Gland || Malignant Thyroid Stimulating Hormone Producing Tumor of the Pituitary || Malignant Thyroid Stimulating Hormone Producing Tumor of the Pituitary Gland || Malignant Thyroid Stimulating Hormone Secreting Neoplasm of Pituitary || Malignant Thyroid Stimulating Hormone Secreting Neoplasm of Pituitary Gland || Malignant Thyroid Stimulating Hormone Secreting Neoplasm of the Pituitary || Malignant Thyroid Stimulating Hormone Secreting Neoplasm of the Pituitary Gland || Malignant Thyroid Stimulating Hormone Secreting Pituitary Gland Neoplasm || Malignant Thyroid Stimulating Hormone Secreting Pituitary Gland Tumor || Malignant Thyroid Stimulating Hormone Secreting Pituitary Neoplasm || Malignant Thyroid Stimulating Hormone Secreting Pituitary Tumor || Malignant Thyroid Stimulating Hormone Secreting Tumor of Pituitary || Malignant Thyroid Stimulating Hormone Secreting Tumor of Pituitary Gland || Malignant Thyroid Stimulating Hormone Secreting Tumor of the Pituitary || Malignant Thyroid Stimulating Hormone Secreting Tumor of the Pituitary Gland || Malignant Thyrotropinoma || Malignant Thyrotropinoma of Pituitary || Malignant Thyrotropinoma of Pituitary Gland || Malignant Thyrotropinoma of the Pituitary || Malignant Thyrotropinoma of the Pituitary Gland || TSH Producing Pituitary Gland Carcinoma || Thyrotropin Producing Pituitary Gland Carcinoma A rare, hormonally functioning or non-functioning pituitary gland adenocarcinoma that produces thyrotropin. Malignant C27886 Type 1 Papillary Renal Cell Carcinoma A papillary renal cell carcinoma characterized by the presence of papillae covered by small cells with scant amount of cytoplasm. The cells are arranged in a single layer on the basement membrane of the papillae. Malignant C27887 Type 2 Papillary Renal Cell Carcinoma A papillary renal cell carcinoma characterized by the presence of papillae covered by cells of a higher nuclear grade as compared to type 1 papillary renal cell carcinoma. The cells have eosinophilic cytoplasm and pseudostratified nuclei. Malignant C7197 Type A Lymphomatoid Papulosis Type A LyP A variant of lymphomatoid papulosis characterized by the presence of scattered anaplastic large lymphocytes, often resembling Reed-Sternberg cells, admixed with acute and chronic inflammatory cells. Uncertain Malignant Potential C7198 Type B Lymphomatoid Papulosis Type B LyP A variant of lymphomatoid papulosis characterized by an epidermotropic infiltrate composed of small atypical cerebriform-like lymphocytes. Uncertain Malignant Potential C7199 Type C Lymphomatoid Papulosis Diffuse Large Cell Type LyP || Diffuse Large Cell Type Lymphomatoid Papulosis || Type C LyP A variant of lymphomatoid papulosis characterized clinically by regressing skin papules, and morphologically by features resembling anaplastic large cell lymphoma. Uncertain Malignant Potential C40145 Type I Endometrial Adenocarcinoma A classification of primary endometrial adenocarcinomas that refers to endometrioid adenocarcinoma and mucinous adenocarcinoma. Malignant C45626 Type I Pleuropulmonary Blastoma A pleuropulmonary blastoma composed of malignant small cells. Sarcomatous features are absent. Malignant C39749 Type II Endometrial Adenocarcinoma A classification of primary endometrial adenocarcinomas that refers to clear cell adenocarcinoma, serous adenocarcinoma, and serous endometrial intraepithelial carcinoma. Malignant C45627 Type II Pleuropulmonary Blastoma A pleuropulmonary blastoma composed of malignant small cells and characterized by the presence of a sarcomatous component. It usually follows an aggressive clinical course. Malignant C45628 Type III Pleuropulmonary Blastoma A pleuropulmonary blastoma characterized by a solid pattern and sarcomatous features. It usually follows an aggressive clinical course. Malignant C27756 Typical Acute Promyelocytic Leukemia Hypergranular Acute Promyelocytic Leukemia Acute Promyelocytic leukemia characterized by the presence of hypergranular promyelocytes and characteristic cells that contain bundles of Auer rods. Malignant C27892 Unclassified Renal Cell Carcinoma A renal cell carcinoma characterized by morphologic features that do not fit easily into one of the other well-defined categories of renal cell carcinoma. Examples of such features include mixtures of morphologic patterns, mucin production, and sarcomatoid morphology. Malignant C3692 Undifferentiated Carcinoma Anaplastic Carcinoma || Carcinoma, Undifferentiated A usually aggressive malignant epithelial neoplasm composed of atypical cells which do not display evidence of glandular, squamous, or transitional cell differentiation. Malignant C4247 Undifferentiated Pleomorphic Sarcoma Storiform-Pleomorphic Malignant Fibrous Histiocytoma || Unclassified Pleomorphic Sarcoma || Unclassified Pleomorphic Sarcoma (Formerly "MFH") || Unclassified Pleomorphic Sarcoma (Formerly "Malignant Fibrous Histiocytoma") || Undifferentiated Pleomorphic Soft Tissue Sarcoma An undifferentiated soft tissue sarcoma characterized by the presence of a pleomorphic malignant cellular infiltrate. It is also known as malignant fibrous histiocytoma. Malignant C66814 Undifferentiated Retinoblastoma A retinoblastoma composed of small, undifferentiated cells. It is often associated with necrotic changes. Malignant C53994 Undifferentiated Stromal Sarcoma An infiltrating high grade mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. Malignant C46073 Unilateral Breast Carcinoma Unilateral Breast Cancer Breast carcinoma of one breast, or one side of the breast. Malignant C8714 Unilateral Retinoblastoma A retinoblastoma that only involves a single eye. Malignant C8173 Upper Gingival Squamous Cell Carcinoma SCC of Upper Gingiva || SCC of Upper Gum || SCC of the Upper Gingiva || SCC of the Upper Gum || Squamous Cell Carcinoma of Upper Gingiva || Squamous Cell Carcinoma of Upper Gum || Squamous Cell Carcinoma of the Upper Gingiva || Squamous Cell Carcinoma of the Upper Gum || Upper Gingival SCC || Upper Gum SCC || Upper Gum Squamous Cell Carcinoma A squamous cell carcinoma of the oral cavity that arises from the upper gingiva. Malignant C8993 Ureter Carcinoma Carcinoma of Ureter || Carcinoma of the Ureter || Ureteral Carcinoma A carcinoma of the ureter. The majority of ureter carcinomas are transitional cell and less frequently squamous cell carcinomas or adenocarcinomas. Malignant C6154 Ureter Squamous Cell Carcinoma Epidermoid Carcinoma of Ureter || Epidermoid Carcinoma of the Ureter || Squamous Cell Carcinoma of Ureter || Squamous Cell Carcinoma of the Ureter || Ureter Epidermoid Carcinoma || Ureteral Epidermoid Carcinoma || Ureteral Squamous Cell Carcinoma A rare squamous cell carcinoma that arises from the ureter. Malignant C4830 Ureter Urothelial Carcinoma Transitional Cell Carcinoma of Ureter || Transitional Cell Carcinoma of the Ureter || Ureter Transitional Cell Carcinoma || Ureteral Transitional Cell Carcinoma || Ureteral Urothelial Cell Carcinoma || Urothelial Cell Carcinoma of Ureter || Urothelial Cell Carcinoma of the Ureter A carcinoma that arises from the transitional epithelium of the ureter. It is associated with tobacco use and usually presents with gross or microscopic hematuria. Malignant C6167 Urethral Adenocarcinoma Adenocarcinoma of Urethra || Adenocarcinoma of the Urethra || Urethra Adenocarcinoma An adenocarcinoma that arises from the male or female urethra. Malignant C9106 Urethral Carcinoma Carcinoma of Urethra || Carcinoma of the Urethra || Urethra Carcinoma || Urethral Cancer A carcinoma that arises from the urethra. Morphologically, the tumors are similar to those described in the bladder. Malignant C6165 Urethral Squamous Cell Carcinoma Epidermoid Carcinoma of Urethra || Epidermoid Carcinoma of the Urethra || Squamous Cell Carcinoma of Urethra || Squamous Cell Carcinoma of the Urethra || Urethra Epidermoid Carcinoma || Urethra Squamous Cell Carcinoma || Urethral Epidermoid Carcinoma A well differentiated, moderately differentiated, or poorly differentiated squamous cell carcinoma that arises from the male or female urethra. Malignant C6166 Urethral Urothelial Carcinoma Transitional Cell Carcinoma of Urethra || Transitional Cell Carcinoma of the Urethra || Urethra Transitional Cell Carcinoma || Urethral Transitional Cell Carcinoma A transitional cell carcinoma that arises from the male or female urethra. Malignant C113239 Uterine Carcinosarcoma, Heterologous Type A carcinosarcoma of the uterus characterized by the presence of sarcomatous elements composed of tissues that are not found in the uterus (e.g., bone, cartilage, skeletal muscle). Malignant C113238 Uterine Carcinosarcoma, Homologous Type A carcinosarcoma of the uterus characterized by the presence of sarcomatous elements that arise from the tissues of the uterus. Malignant C6336 Uterine Corpus Adenosarcoma Adenosarcoma of Body of Uterus || Adenosarcoma of Uterine Body || Adenosarcoma of Uterine Corpus || Adenosarcoma of the Body of Uterus || Adenosarcoma of the Uterine Body || Adenosarcoma of the Uterine Corpus || Body of Uterus Adenosarcoma || Uterine Body Adenosarcoma || Uterine Corpus Mullerian Adenosarcoma || Uterine Corpus Müllerian Adenosarcoma A primary polypoid malignant neoplasm of the uterine corpus characterized by the presence of a sarcomatous mesenchymal component and a benign epithelial component. Patients usually present with abnormal vaginal bleeding. It is considered a low grade malignant neoplasm and may recur following resection. Malignant C9180 Uterine Corpus Carcinosarcoma Carcinosarcoma of Corpus Uteri || Carcinosarcoma of Uterine Body || Carcinosarcoma of Uterine Corpus || Carcinosarcoma of Uterus || Carcinosarcoma of the Corpus Uteri || Carcinosarcoma of the Uterine Body || Carcinosarcoma of the Uterine Corpus || Carcinosarcoma of the Uterus || Corpus Uteri Malignant Mixed Mesodermal Tumor || Malignant Mixed Mesodermal Neoplasm of Uterine Body || Malignant Mixed Mesodermal Neoplasm of Uterine Corpus || Malignant Mixed Mesodermal Neoplasm of Uterus || Malignant Mixed Mesodermal Neoplasm of the Uterine Body || Malignant Mixed Mesodermal Neoplasm of the Uterine Corpus || Malignant Mixed Mesodermal Neoplasm of the Uterus || Malignant Mixed Mesodermal Tumor of Uterine Body || Malignant Mixed Mesodermal Tumor of Uterine Corpus || Malignant Mixed Mesodermal Tumor of Uterus || Malignant Mixed Mesodermal Tumor of the Uterine Body || Malignant Mixed Mesodermal Tumor of the Uterine Corpus || Malignant Mixed Mesodermal Tumor of the Uterus || Mixed Mullerian Sarcoma of Uterus || Mixed Mullerian Sarcoma of the Uterus || Mixed Müllerian Sarcoma of Uterus || Mixed Müllerian Sarcoma of the Uterus || Uterine Body Carcinosarcoma || Uterine Body Malignant Mixed Mesodermal Neoplasm || Uterine Body Malignant Mixed Mesodermal Tumor || Uterine Carcinosarcoma || Uterine Corpus Malignant Mixed Mesodermal (Mullerian) Tumor || Uterine Corpus Malignant Mixed Mesodermal (Müllerian) Tumor || Uterine Corpus Malignant Mixed Mesodermal Neoplasm || Uterine Corpus Malignant Mixed Mesodermal Tumor || Uterine Corpus Malignant Mixed Mullerian Neoplasm || Uterine Corpus Malignant Mixed Mullerian Tumor || Uterine Corpus Malignant Mixed Müllerian Neoplasm || Uterine Corpus Malignant Mixed Müllerian Tumor || Uterine Malignant Mixed Mesodermal Neoplasm || Uterine Malignant Mixed Mesodermal Tumor || Uterine Mixed Mullerian Sarcoma || Uterine Mixed Müllerian Sarcoma An aggressive malignant mixed epithelial and mesenchymal tumor that arises from the uterine corpus. It usually affects elderly postmenopausal women and presents with vaginal bleeding. It is characterized by the presence of a malignant epithelial component that is usually glandular and a sarcomatous component. Malignant C40219 Uterine Corpus Endometrial Stromal Sarcoma Uterine Corpus ESS A uterine corpus sarcoma originating from the endometrial stroma. It is further subdivided into low grade and high grade endometrial stromal sarcoma. Malignant C126998 Uterine Corpus High Grade Endometrial Stromal Sarcoma High Grade ESS || Uterine Corpus High Grade ESS || YWHAE-FAM22 Endometrial Stromal Sarcoma A rare, high grade sarcoma that arises from the endometrial stroma. It is characterized by round cell morphology. It was previously also known as undifferentiated uterine sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. It appears to have a prognosis that falls between low grade endometrial stromal sarcoma and undifferentiated sarcoma. Malignant C3434 Uterine Corpus Leiomyoma Body of Uterus Fibroid || Body of Uterus Leiomyoma || Corpus Uteri Fibroid || Corpus Uteri Leiomyoma || Fibroid of Body of Uterus || Fibroid of Corpus Uteri || Fibroid of Uterine Body || Fibroid of Uterine Corpus || Fibroid of the Body of Uterus || Fibroid of the Corpus Uteri || Fibroid of the Uterine Body || Fibroid of the Uterine Corpus || Leiomyoma of Body of Uterus || Leiomyoma of Corpus Uteri || Leiomyoma of Uterine Body || Leiomyoma of Uterine Corpus || Leiomyoma of the Body of Uterus || Leiomyoma of the Corpus Uteri || Leiomyoma of the Uterine Body || Leiomyoma of the Uterine Corpus || Uterine Body Fibroid || Uterine Body Leiomyoma || Uterine Corpus Fibroid || Uterine Corpus Leiomyomata || Uterine Fibroid A benign smooth muscle neoplasm arising from the body of the uterus. It is characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern. Benign C6340 Uterine Corpus Leiomyosarcoma Body of Uterus Leiomyosarcoma || Corpus Uteri Leiomyosarcoma || Leiomyosarcoma of Body of Uterus || Leiomyosarcoma of Corpus Uteri || Leiomyosarcoma of Uterine Body || Leiomyosarcoma of Uterine Corpus || Leiomyosarcoma of Uterus || Leiomyosarcoma of the Body of Uterus || Leiomyosarcoma of the Corpus Uteri || Leiomyosarcoma of the Uterine Body || Leiomyosarcoma of the Uterine Corpus || Leiomyosarcoma of the Uterus || Uterine Body Leiomyosarcoma An aggressive malignant smooth muscle neoplasm, arising from the uterine corpus. It is characterized by a proliferation of neoplastic spindle cells. Malignant C40223 Uterine Corpus Low Grade Endometrial Stromal Sarcoma JAZF1-SUZ12 Endometrial Stromal Sarcoma || Uterine Corpus Low Grade ESS A rare, indolent, invasive mesenchymal tumor that arises from the endometrial stroma. It is characterized by the presence of a plexiform vasculature, infrequent mitoses, and insignificant cytologic atypia. Late recurrences may occur. Malignant C7981 Vaginal Adenocarcinoma Adenocarcinoma of Vagina || Adenocarcinoma of the Vagina || Vagina Adenocarcinoma An adenocarcinoma arising from the vagina. Morphologic variants include the clear cell, endometrioid, mesonephric, and mucinous adenocarcinoma. Malignant C40260 Vaginal Adenosquamous Carcinoma A carcinoma that arises from the vagina and is characterized by the presence of malignant glandular and malignant squamous epithelial components. Malignant C3917 Vaginal Carcinoma Cancer of Vagina || Cancer of the Vagina || Carcinoma of Vagina || Carcinoma of the Vagina || Vagina Cancer || Vagina Carcinoma || Vaginal Cancer A carcinoma arising from the vaginal epithelium. The majority of vaginal carcinomas are squamous cell carcinomas. Malignant C7736 Vaginal Squamous Cell Carcinoma, Not Otherwise Specified Epidermoid Carcinoma of Vagina || Epidermoid Carcinoma of the Vagina || Epidermoid Cell Carcinoma of Vagina || Epidermoid Cell Carcinoma of the Vagina || Squamous Cell Carcinoma of Vagina || Squamous Cell Carcinoma of the Vagina || Vagina Epidermoid Carcinoma || Vagina Epidermoid Cell Carcinoma || Vagina Squamous Cell Carcinoma || Vaginal Epidermoid Carcinoma || Vaginal Epidermoid Cell Carcinoma || Vaginal Squamous Cell Carcinoma || Vaginal Squamous Cell Carcinoma, NOS A squamous cell carcinoma arising from the vagina. Human papillomavirus infection is associated with the development of vaginal intraepithelial neoplasia and invasive squamous cell carcinoma. Signs and symptoms include painless bleeding, postcoital bleeding, and urinary tract symptoms. Morphologically it resembles squamous cell carcinomas in other anatomic sites. Radiation therapy is the preferred treatment for most cases. The prognosis is related to the stage of the disease. Malignant C27087 Verruca Vulgaris A wart caused by human papillomavirus. Benign C7533 Visual Pathway Astrocytoma An astrocytoma that affects the visual pathway. This condition can be seen in association with neurofibromatosis 1. It is most commonly seen in the pediatric age group. Undetermined C8567 Visual Pathway Glioma Glioma of Visual Pathway || Glioma of the Visual Pathway A glioma that arises from the visual pathway. This condition can be seen in association with neurofibromatosis 1. It is most commonly seen in the pediatric age group. Undetermined C6380 Vulvar Adenocarcinoma Adenocarcinoma of Vulva || Adenocarcinoma of the Vulva An adenocarcinoma that arises from the vulva. Representative examples include Bartholin gland adenocarcinoma, eccrine adenocarcinoma, apocrine adenocarcinoma, and sebaceous carcinoma. Malignant C6381 Vulvar Basal Cell Carcinoma Basal Cell Carcinoma of Vulva || Basal Cell Carcinoma of the Vulva A slow growing, locally infiltrating carcinoma that arises from the vulva. It is characterized by the presence of malignant cells that resemble the basal cells that are present in the epidermis. Malignant C4866 Vulvar Carcinoma Cancer of Vulva || Cancer of the Vulva || Carcinoma of Vulva || Carcinoma of the Vulva || Vulva Cancer || Vulva Carcinoma || Vulvar Cancer A carcinoma arising from the vulva. It usually affects elderly women but has been observed in premenopausal women as well. The most frequently seen histologic type is squamous cell carcinoma. The most common site of involvement is the labia majora. In many cases, the development of vulvar cancer is preceded by condyloma or squamous dysplasia. Malignant C4027 Vulvar Paget Disease Paget Disease of the Vulva || Paget's Disease of Vulva || Paget's Disease of the Vulva || Vulva Paget's Disease || Vulvar Paget's Disease An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising from the vulva. It usually affects post-menopausal women. In approximately 10-20% of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically, it is characterized by the presence of the typical Paget cells which are large, round cells with abundant cytoplasm and prominent nuclei. Malignant C4052 Vulvar Squamous Cell Carcinoma Epidermoid Carcinoma of Vulva || Epidermoid Carcinoma of the Vulva || Epidermoid Cell Carcinoma of Vulva || Epidermoid Cell Carcinoma of the Vulva || Squamous Cell Carcinoma of Vulva || Squamous Cell Carcinoma of the Vulva || Vulva Epidermoid Carcinoma || Vulva Epidermoid Cell Carcinoma || Vulva Squamous Cell Carcinoma || Vulvar Epidermoid Carcinoma || Vulvar Epidermoid Cell Carcinoma An invasive squamous cell carcinoma arising from the vulva. Risk factors include the human papilloma virus and cigarette smoking. Precursor lesions include the vulvar intraepithelial neoplasia, lichen sclerosus with associated squamous cell hyperplasia, and chronic granulomatous vulvar disease such as granuloma inguinale. Symptoms include vulvar pruritus or irritation, discharge, bleeding, and pain. The following morphologic variants have been identified: keratinizing, non-keratinizing, basaloid, warty, verrucous, keratoacanthoma-like, and squamous cell carcinoma with tumor giant cells. Risk factors for recurrence include advanced stage, tumor diameter greater than 2.5 cm, multifocality, capillary-like space involvement, associated vulvar intraepithelial neoplasia grades 2 or 3, and margins of resection involved by tumor. (WHO, 2003) Malignant C80307 Waldenstrom Macroglobulinemia Waldenstrom's Macroglobulinemia || Waldenström Macroglobulinemia Lymphoplasmacytic lymphoma associated with bone marrow involvement and IgM monoclonal gammopathy. Malignant C2854 Warthin Tumor Adenolymphoma || Papillary Cystadenoma Lymphomatosum || Salivary Gland Warthin Tumor || Warthin's Tumor An adenoma characterized by an oncocytic, often papillary, epithelial component, dense lymphoid stroma, and cystic spaces. It occurs primarily in the parotid gland, and is the second most common benign parotid salivary gland tumor. A strong association with smoking has been reported. It typically presents as a painless swelling in the lower portion of the parotid gland. Benign C68635 Well Differentiated Adrenal Cortex Carcinoma An adrenal cortex carcinoma characterized by the presence of morphologic and cytologic features reminiscent of the normal adrenal cortex and absence of sarcomatous or anaplastic features. Malignant C9389 Well Differentiated Leiomyosarcoma Grade I malignant smooth muscle neoplasm. It is characterized by a proliferation of neoplastic spindle cells. Malignant C7635 Well Differentiated Papillary Mesothelial Tumor Benign/Intermediate Mesothelioma || Well Differentiated Papillary Mesothelioma || Well-Differentiated Mesothelial Papillary Neoplasm || Well-Differentiated Mesothelial Papillary Tumor || Well-Differentiated Papillary Mesothelial Tumor || Well-Differentiated Papillary Neoplasm of Mesothelium || Well-Differentiated Papillary Neoplasm of the Mesothelium || Well-Differentiated Papillary Tumor of Mesothelium || Well-Differentiated Papillary Tumor of the Mesothelium A localized or multifocal mesothelioma arising from the peritoneum and less often the pleura. Cases arising from the peritoneum predominantly occur in women. It is characterized by the formation of papillae, covered by a single layer of blunt mesothelial cells. Mitotic figures are not present. There is no evidence of severe cytologic atypia. It has a relatively favorable clinical outcome, compared to malignant mesothelioma. Uncertain Malignant Potential C27905 Well Differentiated Prostate Adenocarcinoma An invasive prostate adenocarcinoma characterized by the presence of well differentiated malignant glandular epithelial components. Malignant C3267 Wilms Tumor Nephroblastoma || Wilms' Tumor An embryonal neoplasm characterized by the presence of epithelial, mesenchymal, and blastema components. The vast majority of cases arise from the kidney. A small number of cases with morphologic features resembling Wilms tumor of the kidney have been reported arising from the ovary and the cervix. Malignant C3011 Yolk Sac Tumor Endodermal Sinus Neoplasm || Endodermal Sinus Tumor || Yolk Sac Neoplasm || Yolk Sac Tumour Site Unspecified A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. It occurs in the infant testis, ovary, sacrococcygeal region, vagina, uterus, prostate, abdomen, liver, retroperitoneum, thorax, and pineal/third ventricle. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). Treatment includes: surgical resection, radiation, and chemotherapy. This tumor is very responsive to chemotherapy regimens that include cisplatinum. Malignant