NCIt Subset Code NCIt Subset Preferred Term NCIt Code NCIt Preferred Term NCIt Synonym NCIt Definiton Display Name C138189 CTS-API Disease Broad Category Terminology C2916 Carcinoma Malignant Epithelioma|Malignant Epithelial Tumor|Carcinoma|Malignant Epithelial Neoplasm|Epithelial Carcinoma 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. Other Carcinoma C138189 CTS-API Disease Broad Category Terminology C2991 Disease or Disorder Disorder|Diseases and Disorders|Disease or Disorder|Disorders|Disease|Diseases Any abnormal condition of the body or mind that causes discomfort, dysfunction, or distress to the person affected or those in contact with the person. The term is often used broadly to include injuries, disabilities, syndromes, symptoms, deviant behaviors, and atypical variations of structure and function. Other Disease C138189 CTS-API Disease Broad Category Terminology C3262 Neoplasm Neoplastic Growth|Neoplasm|Neoplasia A benign or malignant tissue growth resulting from uncontrolled cell proliferation. Benign neoplastic cells resemble normal cells without exhibiting significant cytologic atypia, while malignant cells exhibit overt signs such as dysplastic features, atypical mitotic figures, necrosis, nuclear pleomorphism, and anaplasia. Representative examples of benign neoplasms include papillomas, cystadenomas, and lipomas; malignant neoplasms include carcinomas, sarcomas, lymphomas, and leukemias. Other Neoplasm C138190 CTS-API Disease Main Type Terminology C3167 Acute Lymphoblastic Leukemia Lymphoblastic Leukemia|Precursor Cell Lymphoblastic Leukemia|Precursor Lymphoblastic Leukemia|Acute Lymphoid Leukemia|ALL - Acute Lymphocytic 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. Acute Lymphoblastic Leukemia (ALL) C138190 CTS-API Disease Main Type Terminology C3171 Acute Myeloid Leukemia Acute Myelogenous Leukemia|AML - Acute Myeloid Leukemia|Hematopoeitic - Acute Myleogenous Leukemia (AML)|Acute Myeloid Leukemia|Acute Myeloblastic Leukemia|Acute Myelogenous Leukemias 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). Acute Myeloid Leukemia (AML) C138190 CTS-API Disease Main Type Terminology C9325 Adrenal Cortex Carcinoma Adrenal Cortical Adenocarcinoma|Adrenal Cortex Adenocarcinoma|Carcinoma of the Adrenal Cortex|Adrenal Cortex Carcinoma|Carcinoma of Adrenal Cortex|Adrenocortical Carcinoma|Adrenal Cortical Carcinoma|Adrenal Cortex Cancer 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. Adrenal Cortex Cancer C138190 CTS-API Disease Main Type Terminology 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. Ampulla of Vater Cancer C138190 CTS-API Disease Main Type Terminology C9291 Anal Carcinoma Cancer of the Anus|Carcinoma of Anus|Anal Cancer|Cancer 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). Anal Cancer C138190 CTS-API Disease Main Type Terminology C3088 Angiosarcoma Hemangiosarcoma|Malignant Hemangioendothelioma|Malignant Angioendothelioma 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. C138190 CTS-API Disease Main Type Terminology C9330 Appendix Carcinoma Carcinoma of the Appendix|Carcinoma of Appendix|Appendix Cancer A malignant epithelial neoplasm arising from the appendix. The vast majority of the cases are adenocarcinomas. Appendix Cancer C138190 CTS-API Disease Main Type Terminology C6906 Atypical Teratoid/Rhabdoid Tumor Rhabdoid Neoplasm of CNS|Atypical Teratoid/Rhabdoid Tumor (WHO Grade IV)|Atypical Teratoid/Rhabdoid Tumor|Primary Malignant Rhabdoid Neoplasm of the Brain|Rhabdoid Tumor of Central Nervous System|Malignant Brain Rhabdoid Neoplasm|Malignant Rhabdoid Neoplasm of the Brain|Primary Malignant Brain Rhabdoid Tumor|Rhabdoid Neoplasm of the Central Nervous System|Central Nervous System Rhabdoid Tumor|Rhabdoid Tumor of the CNS|CNS Rhabdoid Tumor|Primary Malignant Brain Rhabdoid Neoplasm|Rhabdoid Tumor of CNS|Primary Malignant Rhabdoid Neoplasm of Brain|Rhabdoid Neoplasm of Central Nervous System|Malignant Brain Rhabdoid Tumor|Malignant Rhabdoid Neoplasm of Brain|Malignant Rhabdoid Tumor of the Brain|Primary Malignant Rhabdoid Tumor of the Brain|Malignant Rhabdoid Tumor of Brain|Rhabdoid Neoplasm of the CNS|Central Nervous System Rhabdoid Neoplasm|CNS Rhabdoid Neoplasm|Primary Malignant Rhabdoid Tumor of Brain|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 INI1 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. C138190 CTS-API Disease Main Type Terminology C27814 Bile Duct Carcinoma Bile Duct Cancer A malignant tumor arising from the epithelium of the intrahepatic or extrahepatic bile duct. Carcinomas that arise from the intrahepatic bile ducts and the hepatic ducts are called cholangiocarcinomas and are almost always adenocarcinomas. Carcinomas that arise from the extrahepatic bile ducts are adenocarcinomas, adenosquamous carcinomas, squamous cell carcinomas, small cell carcinomas, or mucoepidermoid carcinomas. Bile Duct Cancer C138190 CTS-API Disease Main Type Terminology C4912 Bladder Carcinoma Bladder Carcinoma|Bladder Cancer|Carcinoma of the Urinary Bladder|Urinary Bladder Cancer|Urinary Bladder Carcinoma|Carcinoma of Bladder|Cancer of the Bladder|Cancer of the Urinary Bladder|Carcinoma of Urinary Bladder|Carcinoma of the Bladder|Cancer of Urinary Bladder|Cancer of Bladder 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. Bladder Cancer C138190 CTS-API Disease Main Type Terminology C9312 Bone Sarcoma Osseous Sarcoma|Sarcoma of the Bone|Sarcoma of Bone|Skeletal Sarcoma A sarcoma that arises from the bone. Representative examples are osteosarcoma and chondrosarcoma. Bone Sarcoma C138190 CTS-API Disease Main Type Terminology C4872 Breast Carcinoma Mammary Carcinoma|Cancer of the Breast|Carcinoma of Breast|Breast Carcinoma|Breast Cancer|Cancer of Breast|Carcinoma of the Breast 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. Breast Cancer C138190 CTS-API Disease Main Type Terminology C9293 Central Nervous System Neoplasm Tumor of the Central Nervous System|Neoplasm of CNS|Tumor of the CNS|Neoplasm of the Central Nervous System|Tumor of Central Nervous System|Central Nervous System Tumor|CNS Tumor|Tumor of CNS|CNS Neoplasm|Neoplasm of 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. Brain/Spinal Cord Tumor C138190 CTS-API Disease Main Type Terminology C9039 Cervical Carcinoma Carcinoma of the Uterine Cervix|Cervical Cancer|Cancer of the Cervix|Carcinoma of Cervix|Cervix Uteri Carcinoma|Uterine Cervix Cancer|Cancer of the Uterine Cervix|Carcinoma of Uterine Cervix|Carcinoma of the Cervix Uteri|Cervix Carcinoma|Cancer of Cervix|Cervical Carcinoma|Carcinoma of the Cervix|Uterine Cervix Carcinoma|Carcinoma of Cervix Uteri|Cancer of Uterine Cervix|Cervix Cancer A carcinoma arising from either the exocervical squamous epithelium or the endocervical glandular epithelium. The major histologic types of cervical carcinoma are: squamous carcinoma, adenocarcinoma, adenosquamous carcinoma, adenoid cystic carcinoma and undifferentiated carcinoma. Cervical Cancer C138190 CTS-API Disease Main Type Terminology C4436 Cholangiocarcinoma Cholangiocarcinoma A carcinoma that arises from the intrahepatic biliary tree (intrahepatic cholangiocarcinoma) or from the junction, or adjacent to the junction, of the right and left hepatic ducts (hilar cholangiocarcinoma). 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. C138190 CTS-API Disease Main Type Terminology 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. C138190 CTS-API Disease Main Type Terminology 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. Microscopically, chordomas are composed of cells that form cords and lobules, separated by mucoid intercellular tissue. Some of the cells are large (physaliphorous) and have vacuolated cytoplasm and prominent vesicular nuclei. Other tumor cells are small with small nuclei without visible nucleoli. Chordomas tend to recur and may metastasize. The most common sites of metastasis are the skin and bone. C138190 CTS-API Disease Main Type Terminology C2948 Choriocarcinoma 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. Choriocarcinoma C138190 CTS-API Disease Main Type Terminology C4715 Choroid Plexus Carcinoma Cancer of Choroid Plexus|Choroid Plexus Cancer|Carcinoma of the Choroid Plexus|Choroid Plexus Carcinoma|Carcinoma of Choroid Plexus|Cancer of the Choroid Plexus 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) Choroid Plexus Cancer C138190 CTS-API Disease Main Type Terminology C3163 Chronic Lymphocytic Leukemia B-Cell Chronic Lymphogenous Leukemia|Chronic Lymphatic Leukemia|B Cell CLL|B-Cell Chronic Lymphocytic Leukemia|B Cell Chronic Lymphocytic Leukemia|B-Cell Lymphocytic Leukemia|Chronic Lymphogenous Leukemia|B Cell Lymphocytic Leukemia|Chronic B-Cell Lymphocytic Leukemia|Hematopoeitic - Chronic Lymphocytic Leukemia (CLL)|B-Cell CLL|B-Cell Chronic Lymphoid Leukemia 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) Chronic Lymphocytic Leukemia (CLL) C138190 CTS-API Disease Main Type Terminology C3174 Chronic Myelogenous Leukemia, BCR-ABL1 Positive Chronic Granulocytic Leukemia|Chronic Myelogenous Leukemias|Chronic Myelogenous Leukemia|Chronic Myelocytic Leukemia|BCR-ABL Positive Chronic Myelogenous Leukemia|CML - Chronic Myelogenous Leukemia|Hematopoeitic - Chronic Myelocytic Leukemia (CML)|Chronic Myelogenous Leukemia, BCR-ABL1 Positive|Chronic Myeloid Leukemia 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. Chronic Myelogenous Leukemia (CML) C138190 CTS-API Disease Main Type Terminology C3178 Chronic Myelomonocytic Leukemia 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. Chronic Myelomonocytic Leukemia (CMML) C138190 CTS-API Disease Main Type Terminology C4910 Colon Carcinoma Colon Cancer|Carcinoma of Colon|Colonic Carcinoma|Carcinoma of the Colon|Colon Carcinoma A malignant epithelial neoplasm that arises from the colon and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. Colon Cancer C138190 CTS-API Disease Main Type Terminology C2955 Colorectal Carcinoma Carcinoma of Large Intestine|Cancer of the Large Intestine|Carcinoma of the Large Bowel|Large Intestine Carcinoma|Carcinoma of the Large Intestine|Colorectal Cancer|Cancer of Large Bowel|Cancer of the Large Bowel|Carcinoma of Large Bowel|Large Bowel Carcinoma|Cancer of Large Intestine|Large Intestine Cancer|Large Bowel Cancer 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. Colorectal Cancer C138190 CTS-API Disease Main Type Terminology C38661 Composite Lymphoma Coexistence of Hodgkin and non-Hodgkin lymphoma in the same anatomic site. C138190 CTS-API Disease Main Type Terminology C3510 Cutaneous Melanoma Malignant Melanoma of Skin|Cutaneous Melanoma|Skin Melanoma|Malignant Cutaneous Melanoma|Malignant Melanoma of Skin Stage Unspecified|Melanoma of Skin|Skin, Melanoma|Melanoma of the Skin|Malignant Melanoma (of Skin), Stage Unspecified 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. Cutaneous (Skin) Melanoma C138190 CTS-API Disease Main Type Terminology C7109 Distal Bile Duct Carcinoma Distal Biliary Tract Carcinoma|Carcinoma of Distal Biliary Tract|Carcinoma of the Distal Biliary Tract A carcinoma that arises from the common bile duct distal to the insertion of the cystic duct. Distal Bile Duct Cancer C138190 CTS-API Disease Main Type Terminology C2996 Dysgerminoma A malignant germ cell tumor characterized by the presence of a monotonous primitive germ cell population. The neoplastic cells form aggregates and have an abundant pale cytoplasm and uniform nuclei. The aggregates of the germ cells are separated by fibrous septa which contain inflammatory cells, mostly T-lymphocytes. It arises primarily in the ovaries, but can occur both primarily and secondarily at other sites, particularly the central nervous system. It responds to chemotherapy and radiotherapy. Its prognosis is related to the tumor stage. Dysgerminoma C138190 CTS-API Disease Main Type Terminology C3752 Embryonal Carcinoma 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). Embryonal Carcinoma C138190 CTS-API Disease Main Type Terminology C3264 Embryonal Neoplasm A usually malignant neoplasm composed of primitive (immature) tissues that resemble fetal tissues. Medulloblastoma, Ependymoblastoma, Pineoblastoma, and Wilms tumor are representative embryonal neoplasms. --2003 C138190 CTS-API Disease Main Type Terminology C7558 Endometrial Carcinoma Carcinoma of the Endometrium|Carcinoma of Endometrium|Endometrial Carcinoma 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. Endometrial Cancer C138190 CTS-API Disease Main Type Terminology C3017 Ependymoma WHO Grade II Ependymal Neoplasm|Ependymoma|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) C138190 CTS-API Disease Main Type Terminology C3513 Esophageal Carcinoma Esophagus Carcinoma|Esophageal Carcinoma|Cancer of Esophagus|Carcinoma of the Esophagus|Carcinoma of Esophagus|Cancer of the Esophagus|Esophageal Cancer 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. Esophageal Cancer C138190 CTS-API Disease Main Type Terminology C4817 Ewing Sarcoma Ewing's Sarcoma|ES|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. C138190 CTS-API Disease Main Type Terminology C3867 Fallopian Tube Carcinoma Carcinoma of the Fallopian Tube|Carcinoma of Fallopian Tube|Cancer of the Fallopian Tube|Fallopian Tube Cancer|Cancer of Fallopian Tube 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. Fallopian Tube Cancer C138190 CTS-API Disease Main Type Terminology C3844 Gallbladder Carcinoma Cancer of Gallbladder|Carcinoma of the Gallbladder|Cancer of the Gallbladder|Carcinoma of 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. Gallbladder Cancer C138190 CTS-API Disease Main Type Terminology 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. C138190 CTS-API Disease Main Type Terminology C4911 Gastric Carcinoma Carcinoma of Stomach|Gastric Cancer|Cancer of the Stomach|Stomach Carcinoma|Stomach Cancer|Carcinoma of the Stomach|Gastric Carcinoma|Cancer of Stomach A malignant epithelial tumor of the stomach mucosa. The vast majority of gastric carcinomas are adenocarcinomas, arising from the gastric glandular epithelium. Gastric (Stomach) Cancer C138190 CTS-API Disease Main Type Terminology 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. Gastrointestinal Stromal Tumor (GIST) C138190 CTS-API Disease Main Type Terminology C3708 Germ Cell Tumor Neoplasm of Germ Cell|Germ Cell Neoplasm|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. Germ Cell Tumor C138190 CTS-API Disease Main Type Terminology C3753 Germinoma Germinoma A malignant germ cell tumor arising in the central nervous system. It is characterized by the presence of primitive, large malignant germ cells and lymphocytes. Germinoma C138190 CTS-API Disease Main Type Terminology C4699 Gestational Trophoblastic Tumor Gestational Trophoblastic Disease A diverse group of pregnancy-related tumors characterized by excessive proliferation of trophoblasts. Representative examples include hydatidiform mole, gestational choriocarcinoma, and placental site trophoblastic tumor. Gestational Trophoblastic Tumor C138190 CTS-API Disease Main Type Terminology C3058 Glioblastoma Grade IV Astrocytic Neoplasm|Glioblastoma|Spongioblastoma Multiforme|Grade IV Astrocytic Tumor|WHO Grade IV Glioma|GBM (Glioblastoma)|Glioblastoma Multiforme 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) C138190 CTS-API Disease Main Type Terminology C3059 Glioma Neuroglial Tumor|Glial Neoplasm|Neuroglial Neoplasm|Tumor of Neuroglia|Glioma|Tumor of the Neuroglia|Glial Tumor|Neoplasm of Neuroglia|Neoplasm 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. C138190 CTS-API Disease Main Type Terminology C35850 Head and Neck Carcinoma Carcinoma of Head and Neck|Head and Neck Carcinoma|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. Head and Neck Cancer C138190 CTS-API Disease Main Type Terminology C27134 Hematopoietic and Lymphoid Cell Neoplasm Hematologic Neoplasm|Hematologic Malignancy|Hematopoietic Tumor|Hematopoietic and Lymphoid Neoplasms|Hematopoietic Neoplasms including Lymphomas|Hematopoietic Neoplasm|Hematopoietic Cancer|Malignant Hematologic Neoplasm|Hematological Tumor|Hematopoietic Cell Tumor|Malignant Hematopoietic Neoplasm|Hematopoietic, Including Myeloma|Hematologic Cancer|Hematological Neoplasm A neoplasm arising from hematopoietic cells found in the bone marrow, peripheral blood, lymph nodes and spleen (organs of the hematopoietic system). Hematopoietic cell neoplasms can also involve other anatomic sites (e.g. central nervous system, gastrointestinal tract), either by metastasis, direct tumor infiltration, or neoplastic transformation of extranodal lymphoid tissues. The commonest forms are the various types of leukemia, Hodgkin and non-Hodgkin lymphomas, myeloproliferative neoplasms, and myelodysplastic syndromes. C138190 CTS-API Disease Main Type Terminology C3728 Hepatoblastoma Pediatric Hepatoblastoma|Pediatric Embryonal Hepatoma 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. Hepatoblastoma C138190 CTS-API Disease Main Type Terminology C3099 Hepatocellular Carcinoma Liver Cell Cancer (Hepatocellular Carcinoma)|Carcinoma of Liver Cells|Hepatocellular Carcinoma|Hepatoma|Primary Carcinoma of the Liver Cells|Liver Cell Carcinoma|Carcinoma of the Liver Cells|Primary Carcinoma of 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. Hepatocellular Cancer C138190 CTS-API Disease Main Type Terminology C36077 Hilar Cholangiocarcinoma Klatskin Tumor|Hilar CC A cholangiocarcinoma that arises from the junction, or adjacent to the junction, of the right and left hepatic ducts. C138190 CTS-API Disease Main Type Terminology 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. Hodgkin Lymphoma C138190 CTS-API Disease Main Type Terminology C9465 Hypopharyngeal Carcinoma Cancer of the Hypopharynx|Carcinoma of Hypopharynx|Cancer of Hypopharynx|Hypopharyngeal Cancer|Carcinoma of the Hypopharynx Carcinoma, predominantly squamous cell, arising from the epithelial cells of the hypopharynx. Hypopharyngeal Throat Cancer C138190 CTS-API Disease Main Type Terminology C35417 Intrahepatic Cholangiocarcinoma Intrahepatic Carcinoma of Bile Duct|Intrahepatic Carcinoma of the Bile Duct|Intrahepatic Bile Duct Carcinoma 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. Intrahepatic Cholangiocarcinoma (Bile Duct Cancer) C138190 CTS-API Disease Main Type Terminology 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). C138190 CTS-API Disease Main Type Terminology C9384 Kidney Carcinoma Renal Carcinoma|Kidney Cancer|Renal Cancer 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. Kidney (Renal) Cancer C138190 CTS-API Disease Main Type Terminology C4855 Laryngeal Carcinoma Laryngeal Cancer|Carcinoma of the Larynx|Cancer of Larynx|Larynx Carcinoma|Carcinoma of Larynx|Cancer of the Larynx Carcinoma that arises from the laryngeal epithelium. More than 90% of laryngeal carcinomas are squamous cell carcinomas. The remainder are adenoid cystic carcinomas, mucoepidermoid carcinomas and carcinomas with neuroendocrine differentiation. Laryngeal Throat Cancer C138190 CTS-API Disease Main Type Terminology C3158 Leiomyosarcoma 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. C138190 CTS-API Disease Main Type Terminology C3161 Leukemia 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. Leukemia C138190 CTS-API Disease Main Type Terminology C4896 Leukemia in Remission Leukemia (in Remission) History of leukemia after therapy with no evidence on the peripheral blood or bone marrow of leukemia (normalization of all hematologic parameters). C138190 CTS-API Disease Main Type Terminology C9315 Lip and Oral Cavity Carcinoma Lip and Oral Cavity Cancer|Oral Carcinoma|Oral Cancer 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. Lip and Oral Cavity Cancer C138190 CTS-API Disease Main Type Terminology C3194 Liposarcoma Liposarcoma Not Otherwise Specified 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. C138190 CTS-API Disease Main Type Terminology C7927 Liver and Intrahepatic Bile Duct Carcinoma Cancer of the Liver and Intrahepatic Biliary Tract|Cancer of the Liver|Cancer of Liver and Intrahepatic Biliary Tract|Liver and Intrahepatic Biliary Tract Carcinoma|Primary Liver Carcinoma|Cancer of Liver|Liver and Intrahepatic Biliary Tract Cancer|Liver Cancer|Hepatic Cancer|Liver and Intrahepatic Bile Duct Cancer A carcinoma that arises from the hepatocytes or intrahepatic bile ducts. The main subtypes are hepatocellular carcinoma (hepatoma) and cholangiocarcinoma. Liver Cancer C138190 CTS-API Disease Main Type Terminology C4878 Lung Carcinoma Cancer of the Lung|Carcinoma of Lung|Carcinoma of the Lung|Lung Cancer|Cancer of Lung 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. Lung Cancer C138190 CTS-API Disease Main Type Terminology C7539 Lymphoid Leukemia Lymphogenous Leukemia|Lymphocytic Leukemia A malignant lymphocytic neoplasm of B-cell or T-cell lineage involving primarily the bone marrow and the peripheral blood. This category includes precursor or acute lymphoblastic leukemias and chronic leukemias. Lymphoid Leukemia C138190 CTS-API Disease Main Type Terminology C3208 Lymphoma Malignant Lymphoma|Lymphoma|Lymphoma (Hodgkin and Non-Hodgkin)|Lymphoma (Hodgkin's and Non-Hodgkin's) 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. Lymphoma C138190 CTS-API Disease Main Type Terminology C4016 Malignant Bone Neoplasm Malignant Osseous Tumor|Malignant Neoplasm of the Bone|Malignant Tumor of Bone|Osseous Cancer|Malignant Tumor of the Bone|Malignant Osseous Neoplasm|Cancer of Bone|Malignant Neoplasm of Bone|Malignant Bone Tumor|Bone Cancer|Cancer of the Bone A primary or metastatic malignant neoplasm affecting the bone or articular cartilage. Bone Cancer C138190 CTS-API Disease Main Type Terminology C4767 Malignant Eye Neoplasm Malignant Neoplasm of the Eye|Cancer of Eye|Eye Cancer|Malignant Neoplasm of Eye|Malignant Tumor of Eye|Eye Neoplasm, Malignant|Cancer of the Eye|Malignant Ocular Neoplasm|Malignant Tumor of the Eye|Malignant Ocular Tumor|Malignant Eye Tumor A primary or metastatic malignant neoplasm involving the structures of the eye (conjunctiva, cornea, uvea, retina), the lacrimal gland, and the eyelid. Representative examples include melanoma, carcinoma, lymphoma, and retinoblastoma. Eye Cancer C138190 CTS-API Disease Main Type Terminology C9063 Malignant Testicular Germ Cell Tumor Malignant Germ Cell Neoplasm of Testis|Malignant Germ Cell Tumor of the Testis|Malignant Germ Cell Tumor of Testis|Malignant Testicular Germ Cell Neoplasm|Testicular Germ Cell Cancer|Malignant Germ Cell Neoplasm of the Testis|Malignant Testicular Germ Cell Tumor|Testicular 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. Testicular Cancer C138190 CTS-API Disease Main Type Terminology C3224 Melanoma 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. Melanoma C138190 CTS-API Disease Main Type Terminology C3230 Meningioma 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) Meningioma C138190 CTS-API Disease Main Type Terminology C3234 Mesothelioma A usually malignant and aggressive neoplasm of the mesothelium which is often associated with exposure to asbestos. Mesothelioma C138190 CTS-API Disease Main Type Terminology C4290 Mixed Germ Cell Tumor Mixed Germ Cell Tumor|Mixed Germ Cell Neoplasm|Combined Germ Cell Tumor|Combined Germ Cell Neoplasm A malignant germ cell tumor characterized by the presence of at least two different germ cell components. The different germ cell 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. Mixed Germ Cell Tumor C138190 CTS-API Disease Main Type Terminology C3247 Myelodysplastic Syndrome Smoldering Leukemia|Myelodysplastic Neoplasm|Hematopoeitic - Myelodysplastic Syndrome (MDS)|Oligoblastic Leukemia|Myelodysplastic Syndrome/Neoplasm|Myelodysplasia|Myelodysplastic Syndrome|Dysmyelopoietic Syndrome 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) C138190 CTS-API Disease Main Type Terminology C3172 Myeloid Leukemia Myelocytic Leukemia|Myelogenous Leukemia A clonal proliferation of myeloid cells and their precursors in the bone marrow, peripheral blood, and spleen. When the proliferating cells are immature myeloid cells and myeloblasts, it is called acute myeloid leukemia. When the proliferating myeloid cells are neutrophils, it is called chronic myelogenous leukemia. C138190 CTS-API Disease Main Type Terminology C9290 Myeloid Neoplasm Myeloid Malignancy|Myeloid Tumor Proliferation of myeloid cells originating from a primitive stem cell. C138190 CTS-API Disease Main Type Terminology C54293 Nasal Cavity and Paranasal Sinus Carcinoma Sinonasal Carcinoma A carcinoma arising from the nasal cavity or paranasal sinuses. Nasal Cavity and Paranasal Sinus Cancer C138190 CTS-API Disease Main Type Terminology C3871 Nasopharyngeal Carcinoma Cancer of the Nasopharynx|Carcinoma of Nasopharynx|Cancer of Nasopharynx|Nasopharyngeal Cancer|Carcinoma of the Nasopharynx A carcinoma arising from the nasopharyngeal epithelium. It includes the following types: keratinizing squamous cell carcinoma, nonkeratinizing carcinoma (differentiated and undifferentiated), basaloid squamous cell carcinoma, and papillary adenocarcinoma. Nasopharyngeal Throat Cancer C138190 CTS-API Disease Main Type Terminology C54705 Neoplastic Syndrome Tumor Syndrome A broad classification for disorders in which the development of neoplasms typically occur in association with a characteristic set of signs or symptoms. These disorders may be inherited or acquired. Cancer-Related Syndrome C138190 CTS-API Disease Main Type Terminology C3268 Nervous System Neoplasm Nervous System Tumour|Tumor of Nervous System|Neoplasm of the Nervous System|Tumor of the Nervous System|Nervous System Neoplasms|Neoplasm of Nervous System|Nervous System Tumor A benign or malignant, primary or metastatic neoplasm involving the central or peripheral nervous system. C138190 CTS-API Disease Main Type Terminology C3270 Neuroblastoma Neuroblastoma (Schwannian Stroma-Poor)|Neuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation. Neuroblastoma C138190 CTS-API Disease Main Type Terminology C3773 Neuroendocrine Carcinoma A malignant neuroendocrine neoplasm composed of cells containing secretory granules that stain positive for NSE and chromogranin. The neoplastic cells are often round and form clusters or trabecular sheets. Representative examples are small cell carcinoma, large cell neuroendocrine carcinoma, and Merkel cell carcinoma. Neuroendocrine Cancer C138190 CTS-API Disease Main Type Terminology C3809 Neuroendocrine Neoplasm A benign or malignant neoplasm composed of cells of neuroendocrine origin. Representative examples include paraganglioma, carcinoid tumor, and neuroendocrine carcinoma. C138190 CTS-API Disease Main Type Terminology C8711 Non-Cutaneous Melanoma Extracutaneous Melanoma Melanoma is a malignant tumor of melanocytes, cells that are derived from the neural crest. Although most melanomas arise in the skin, they also may arise from mucosal surfaces or at other sites to which neural crest cells migrate. (PDQ) C138190 CTS-API Disease Main Type Terminology C3211 Non-Hodgkin Lymphoma Non-Hodgkin's Lymphoma (NHL)|Non-Hodgkin's Lymphoma 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. Non-Hodgkin Lymphoma C138190 CTS-API Disease Main Type Terminology C2926 Non-Small Cell Lung Carcinoma Non Small Cell Lung Cancer NOS|NSCLC - Non-Small Cell Lung Cancer|Non-Small Cell Carcinoma of Lung|Non-Small Cell Carcinoma of the Lung|Non-Small Cell Cancer of Lung|Non-Small Cell Lung Cancer|Non-Small Cell Cancer of the Lung 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. Non-Small Cell Lung Cancer C138190 CTS-API Disease Main Type Terminology C8990 Oral Cavity Carcinoma Carcinoma of Mouth|Carcinoma of the Oral Cavity|Carcinoma of 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. Oral Cavity Cancer C138190 CTS-API Disease Main Type Terminology C9105 Oropharyngeal Carcinoma Oropharynx Carcinoma|Cancer of Oropharynx|Carcinoma of the Oropharynx|Carcinoma of Oropharynx|Cancer of the Oropharynx|Oropharyngeal Cancer Carcinoma, predominantly squamous cell, arising from the epithelial cells of the oropharynx. Oropharyngeal Throat Cancer C138190 CTS-API Disease Main Type Terminology 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. Osteosarcoma C138190 CTS-API Disease Main Type Terminology C4908 Ovarian Carcinoma Carcinoma of the Ovary|Ovarian Carcinoma|Carcinoma of 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. --2002 Ovarian Cancer C138190 CTS-API Disease Main Type Terminology C7073 Paget Disease Paget's Cell Neoplasm|Paget Cell Neoplasm|Paget Disease|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. C138190 CTS-API Disease Main Type Terminology C3850 Pancreatic Carcinoma Pancreas Cancer|Cancer of Pancreas|Pancreas Carcinoma|Pancreatic Cancer|Pancreatic Carcinoma|Exocrine Pancreas Carcinoma|Cancer of the Pancreas|Carcinoma of Pancreas|Carcinoma of the Pancreas A carcinoma arising from the exocrine pancreas. The overwhelming majority of pancreatic carcinomas are adenocarcinomas. Pancreatic Cancer C138190 CTS-API Disease Main Type Terminology C4906 Parathyroid Gland Carcinoma Adenocarcinoma of Parathyroid Gland|Cancer of Parathyroid Gland|Adenocarcinoma of the Parathyroid Gland|Carcinoma of Parathyroid Gland|Cancer of the Parathyroid Gland|Parathyroid Cancer|Parathyroid Adenocarcinoma|Carcinoma of the Parathyroid Gland|Adenocarcinoma of Parathyroid|Adenocarcinoma of the Parathyroid|Parathyroid Carcinoma|Carcinoma of Parathyroid|Parathyroid Gland Cancer|Parathyroid Gland Adenocarcinoma|Carcinoma of the Parathyroid 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. Parathyroid Gland Cancer C138190 CTS-API Disease Main Type Terminology C9061 Penile Carcinoma Carcinoma of the Penis|Carcinoma of Penis|Cancer of the Penis|Cancer of 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. Penile Cancer C138190 CTS-API Disease Main Type Terminology C9466 Pharyngeal Carcinoma Pharynx Carcinoma|Carcinoma of Pharynx|Cancer of the Pharynx|Carcinoma of the Pharynx|Pharyngeal Cancer|Cancer of Pharynx Carcinoma, predominantly squamous cell, arising from the epithelial cells of the pharynx. Pharyngeal Throat Cancer C138190 CTS-API Disease Main Type Terminology C9344 Pineoblastoma Primitive Neuroectodermal Neoplasm of the Pineal Gland|Pineoblastoma|Pineal PNET|PNET of Pineal Gland|Pineal Primitive Neuroectodermal Neoplasm|Pineal Gland Primitive Neuroectodermal Neoplasm|Pineoblastoma (WHO Grade IV)|PNET of the Pineal Gland|Pineal Primitive Neuroectodermal Tumor|Primitive Neuroectodermal Neoplasm of Pineal Gland|Primitive Neuroectodermal Tumor of the Pineal Gland|Pineal Gland Primitive Neuroectodermal Tumor|Pineal Gland PNET|Primitive Neuroectodermal Tumor of 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. C138190 CTS-API Disease Main Type Terminology C4536 Pituitary Gland Carcinoma Cancer of the Pituitary Gland|Carcinoma of Pituitary Gland|Pituitary Carcinoma|Cancer of Pituitary|Carcinoma of the Pituitary Gland|Carcinoma of the Pituitary|Pituitary Gland Adenocarcinoma|Pituitary Gland Cancer|Carcinoma of Pituitary|Cancer of the Pituitary|Cancer of Pituitary Gland 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. Pituitary Gland Cancer C138190 CTS-API Disease Main Type Terminology C3242 Plasma Cell Myeloma Myeloma|Plasma Cell Myeloma|Multiple 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) Multiple Myeloma/Plasma Cell Myeloma C138190 CTS-API Disease Main Type Terminology C4665 Plasma Cell Neoplasm Plasmacytic Tumour|Plasma Cell Dyscrasia|Plasmacytic Tumor|Plasmacytic Neoplasm|Plasma Cell Tumor A clonal proliferation of immunoglobulin-secreting plasma cells. This category includes plasma cell myeloma, plasma cell leukemia, plasmacytoma, monoclonal immunoglobulin deposition disease, and monoclonal gammopathy of undetermined significance. C138190 CTS-API Disease Main Type Terminology 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) C138190 CTS-API Disease Main Type Terminology 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. C138190 CTS-API Disease Main Type Terminology 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. Primary Peritoneal Cancer C138190 CTS-API Disease Main Type Terminology C3716 Primitive Neuroectodermal Tumor Neuroepithelioma|Primitive Neuroectodermal Neoplasm|Neuroectodermal Neoplasm|Neuroectodermal Tumor|Primitive Neuroectodermal Tumor A malignant neoplasm that originates in the neuroectoderm. The neuroectoderm constitutes the portion of the ectoderm of the early embryo that gives rise to the central and peripheral nervous systems and includes some glial cell precursors. Primitive Neuroectodermal Tumor (PNET) C138190 CTS-API Disease Main Type Terminology C4863 Prostate Carcinoma Carcinoma of the Prostate|Prostate Carcinoma|Cancer of Prostate|Cancer of the Prostate|Carcinoma of 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. -- 2002 Prostate Cancer C138190 CTS-API Disease Main Type Terminology C9382 Rectal Carcinoma Cancer of Rectum|Carcinoma of the Rectum|Rectal Cancer|Carcinoma of Rectum|Cancer of the Rectum A malignant epithelial neoplasm that arises from the rectum and invades through the muscularis mucosa into the submucosa. The vast majority are adenocarcinomas. Rectal Cancer C138190 CTS-API Disease Main Type Terminology C9385 Renal Cell Carcinoma Kidney Adenocarcinoma|Renal Cell Carcinoma, Stage Unspecified|Adenocarcinoma of the Kidney|Renal Cell Carcinoma|Renal Cell Adenocarcinoma|Adenocarcinoma of Kidney|Renal Cell Cancer 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. Renal Cell Cancer C138190 CTS-API Disease Main Type Terminology C7515 Renal Pelvis and Ureter Carcinoma Renal Pelvis and Ureter Cancer Kidney Renal Pelvis and Ureter Cancer C138190 CTS-API Disease Main Type Terminology C6142 Renal Pelvis Carcinoma Carcinoma of the Renal Pelvis|Kidney Pelvis Carcinoma|Carcinoma of the Kidney Pelvis|Carcinoma of Renal Pelvis|Carcinoma of Kidney Pelvis A carcinoma arising in the renal pelvis. The majority of renal pelvis carcinomas are transitional cell and less frequently squamous cell carcinomas. Kidney Renal Pelvis Cancer C138190 CTS-API Disease Main Type Terminology 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. C138190 CTS-API Disease Main Type Terminology C7352 Retroperitoneal Carcinoma Carcinoma of the Retroperitoneum|Carcinoma of Retroperitoneum A rare carcinoma that arises from the retroperitoneal space. Retroperitoneal Cancer C138190 CTS-API Disease Main Type Terminology 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 anatomical sites. C138190 CTS-API Disease Main Type Terminology 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. Salivary Gland Cancer C138190 CTS-API Disease Main Type Terminology C9118 Sarcoma Sarcoma of Soft Tissue and Bone|Sarcoma|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. C138190 CTS-API Disease Main Type Terminology 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. Seminoma C138190 CTS-API Disease Main Type Terminology C4914 Skin Carcinoma Carcinoma of the Skin|Non-Melanoma Skin Cancer|Skin Carcinoma|Non-Melanoma Cancer of the Skin|Carcinoma of Skin|Non-Melanoma Cancer of Skin|Skin Cancer, Non-Melanoma A carcinoma that arises from the skin. Representative examples are basal cell carcinoma and squamous cell carcinoma. Non-Melanoma Skin Cancer C138190 CTS-API Disease Main Type Terminology C4917 Small Cell Lung Carcinoma Small Cell Carcinoma of Lung|Small Cell Neuroendocrine Carcinoma of Lung|Small Cell Carcinoma of the Lung|Small Cell Neuroendocrine Carcinoma of the Lung|Small Cell Lung Cancer|Lung Small Cell Neuroendocrine Carcinoma 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. Small Cell Lung Cancer C138190 CTS-API Disease Main Type Terminology C7724 Small Intestinal Carcinoma Cancer of Small Bowel|Cancer of the Small Bowel|Carcinoma of Small Bowel|Small Bowel Carcinoma|Carcinoma of Small Intestine|Small Intestine Cancer|Carcinoma of the Small Bowel|Small Bowel Cancer|Carcinoma of the Small Intestine A carcinoma arising from the small intestine. The vast majority are adenocarcinomas. The remaining cases are adenosquamous, squamous, or undifferentiated carcinomas. Small Intestinal Cancer C138190 CTS-API Disease Main Type Terminology C9306 Soft Tissue Sarcoma Connective Tissue Sarcoma|Sarcoma of Soft Tissue|Sarcoma of the Soft Tissue A malignant neoplasm arising from muscle tissue, adipose tissue, blood vessels, fibrous tissue, or other supportive tissues excluding the bones. Soft Tissue Sarcoma C138190 CTS-API Disease Main Type Terminology 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 mature or immature. Mature teratomas are composed of well differentiated, adult-type tissues. Immature teratomas are composed of immature, fetal-type tissues. Testicular teratomas in children follow a benign clinical course. Mature ovarian teratomas without a fetal-type component have an excellent outcome. The prognosis of immature ovarian teratomas is related to the grade and stage of the tumor. Teratoma C138190 CTS-API Disease Main Type Terminology 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 (WHO-1999). It includes morphologic variants derived from purely epithelial cells, as well as from cells with neuroendocrine differentiation. C138190 CTS-API Disease Main Type Terminology 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. Thymoma C138190 CTS-API Disease Main Type Terminology C4815 Thyroid Gland Carcinoma Carcinoma of the Thyroid|Thyroid Carcinoma|Thyroid Gland Carcinoma|Carcinoma of Thyroid|Cancer of the Thyroid|Head and Neck Cancer, Thyroid|Thyroid Gland Cancer|Cancer of Thyroid|Carcinoma of the Thyroid Gland|Carcinoma of Thyroid Gland|Thyroid Cancer A carcinoma arising from the thyroid gland. It is usually an adenocarcinoma and includes the following main subtypes: follicular, papillary, medullary, poorly differentiated, and anaplastic. Thyroid Gland Cancer C138190 CTS-API Disease Main Type Terminology 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. Trophoblastic Tumor C138190 CTS-API Disease Main Type Terminology 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. --2003 Ureter Cancer C138190 CTS-API Disease Main Type Terminology C9106 Urethral Carcinoma Carcinoma of the Urethra|Carcinoma of Urethra|Urethra Carcinoma|Urethral Cancer Malignant epithelial neoplasm arising from the urethra (lower urinary tract). Urethral Cancer C138190 CTS-API Disease Main Type Terminology C61574 Uterine Corpus Cancer Cancer of the Corpus Uteri|Endometrial Cancer|Corpus Uteri Cancer A malignant neoplasm arising from the uterine corpus. This category includes endometrial carcinoma and carcinosarcoma. Uterine Corpus Cancer C138190 CTS-API Disease Main Type Terminology C6339 Uterine Corpus Sarcoma Sarcoma of the Uterine Body|Sarcoma of the Uterine Corpus|Sarcoma of the Uterus|Body of Uterus Sarcoma|Uterine Sarcoma|Uterus Sarcoma|Corpus Uteri Sarcoma|Sarcoma of Body of Uterus|Sarcoma of the Body of Uterus|Sarcoma of Uterus|Uterine Body Sarcoma|Sarcoma of Corpus Uteri|Sarcoma of Uterine Body|Sarcoma of the Corpus Uteri|Sarcoma of Uterine Corpus A malignant mesenchymal neoplasm arising from the wall of the uterine corpus (uterine body). The most representative examples are leiomyosarcoma and endometrial stromal sarcoma. Uterine Sarcoma C138190 CTS-API Disease Main Type Terminology C3917 Vaginal Carcinoma Carcinoma of Vagina|Cancer of the Vagina|Vagina Cancer|Carcinoma of the Vagina|Cancer of Vagina|Vagina Carcinoma|Vaginal Cancer A carcinoma arising from the vaginal epithelium. The majority of vaginal carcinomas are squamous cell carcinomas. Vaginal Cancer C138190 CTS-API Disease Main Type Terminology C4866 Vulvar Carcinoma Carcinoma of the Vulva|Vulvar Cancer|Vulva Cancer|Vulva Carcinoma|Cancer of the Vulva|Carcinoma of Vulva|Cancer of Vulva 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. Vulvar Cancer C138190 CTS-API Disease Main Type Terminology C3267 Wilms Tumor 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. C138190 CTS-API Disease Main Type Terminology C3011 Yolk Sac Tumor Yolk Sac Tumour Site Unspecified|Yolk Sac Tumor|Endodermal Sinus Neoplasm|Endodermal Sinus Tumor|Yolk Sac Neoplasm 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. Yolk Sac Tumor