A C35710 Triple A Syndrome Triple A Syndrome 3A syndrome|Achalasia-Addisonianism-Alacrima Syndrome|Allgrove Syndrome|Triple-A syndrome An autosomal recessive condition caused by mutation(s) in the AAAS gene encoding the protein ALADIN, and characterized by primary adrenal insufficiency, esophageal dysmotility, and absence of tear production. An autosomal recessive condition caused by mutation(s) in the AAAS gene encoding the protein ALADIN, and characterized by primary adrenal insufficiency, esophageal dysmotility, and absence of tear production. C28193 Syndrome C90259 Pediatric Terminology D C131005 Triple A Syndrome Triple A Syndrome 3A syndrome|Achalasia-Addisonianism-Alacrima Syndrome|Allgrove Syndrome|Triple-A syndrome An autosomal recessive condition caused by mutation(s) in the AAAS gene encoding the protein ALADIN, and characterized by primary adrenal insufficiency, esophageal dysmotility, and absence of tear production. An autosomal recessive condition caused by mutation(s) in the AAAS gene encoding the protein ALADIN, and characterized by primary adrenal insufficiency, esophageal dysmotility, and absence of tear production. C28193 Syndrome C90259 Pediatric Terminology C C69142 Childhood Classic Hodgkin Lymphoma Childhood Classical Hodgkin Lymphoma A classic Hodgkin lymphoma that occurs during childhood. A classical Hodgkin lymphoma that occurs during childhood. Four subtypes are included: mixed cellularity, nodular sclerosis, lymphocyte-depleted, lymphocyte-rich. C7714 Childhood Hodgkin Lymphoma C90259 Pediatric Terminology C C7054 Childhood Lymphocyte-Rich Classic Hodgkin Lymphoma Childhood Lymphocyte-Rich Classical Hodgkin Lymphoma Lymphocyte-rich classic Hodgkin lymphoma occurring in childhood. A lymphocyte-rich, classical Hodgkin lymphoma that occurs during childhood. C69142 Childhood Classical Hodgkin Lymphoma C90259 Pediatric Terminology C C8061 Childhood Lymphocyte-Depleted Classic Hodgkin Lymphoma Childhood Lymphocyte-Depleted Classical Hodgkin Lymphoma Lymphocyte-depleted classic Hodgkin lymphoma occurring in childhood. A lymphocyte-depleted, classical Hodgkin lymphoma that occurs during childhood. C69142 Childhood Classical Hodgkin Lymphoma C90259 Pediatric Terminology C C8062 Childhood Nodular Sclerosis Classic Hodgkin Lymphoma Childhood Nodular Sclerosis Classical Hodgkin Lymphoma Nodular sclerosis classic Hodgkin lymphoma occurring in childhood. A nodular sclerosis, classical Hodgkin lymphoma that occurs during childhood. C69142 Childhood Classical Hodgkin Lymphoma C90259 Pediatric Terminology C C8063 Childhood Mixed Cellularity Classic Hodgkin Lymphoma Childhood Mixed Cellularity Classical Hodgkin Lymphoma Mixed cellularity classic Hodgkin lymphoma occurring in childhood. A mixed cellularity, classical Hodgkin lymphoma that occurs during childhood. C69142 Childhood Classical Hodgkin Lymphoma C90259 Pediatric Terminology C C82403 Acute Myeloid Leukemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A Acute Myeloid Leukemia with t(9;11)(p22;q23); MLLT3-MLL AML with t(9;11)(p22;q23); MLLT3-MLL An acute myeloid leukemia associated with t(9;11)(p21.3;q23.3) and MLLT3-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. An acute myeloid leukemia associated with t(9;11)(p22;q23) and MLLT3 (AF9)-MLL fusion protein expression. t(9;11) occurs in 10% of children with AML. C7175 Acute Myeloid Leukemia with Recurrent Genetic Abnormalities C90259 Pediatric Terminology C C82427 Acute Myeloid Leukemia (Megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 Acute Myeloid Leukemia (Megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1 AML (Megakaryoblastic) with t(1;22)(p13;q13); 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. An acute myeloid leukemia typically showing megakaryocytic maturation and associated with t(1;22)(p13;q13), resulting in the expression of RBM15-MKL1 fusion protein. C7175 Acute Myeloid Leukemia with Recurrent Genetic Abnormalities C90259 Pediatric Terminology C C4348 Nonfunctional Pituitary Gland Adenoma Non-Functioning Pituitary Gland Adenoma A hormone producing or non-producing pituitary gland adenoma not associated with a hormonal syndrome. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C45928 Mammosomatotroph Adenoma Somatolactotrope Adenoma Mammosomatotrope Adenoma|Somatomammotrope 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. A non-malignant pituitary tumor 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. C127194 Growth Hormone and Prolactin-Producing Adenoma C90259 Pediatric Terminology C C121678 Nonfunctional Pituitary Gland Microadenoma Nonfunctional Pituitary Microadenoma A pituitary gland microadenoma not associated with a hormonal syndrome. A non-malignant pituitary tumor with diameter less than 10 millimeters that does not secrete pituitary hormone(s). C4348 C90259 Pediatric Terminology C C121721 Nonfunctional Pituitary Gland Macroadenoma Nonfunctional Pituitary Macroadenoma A pituitary gland macroadenoma not associated with a hormonal syndrome. A non-malignant pituitary tumor with diameter 10 millimeters or greater that does not secrete pituitary hormone(s). C4348 C90259 Pediatric Terminology C C127194 Somatotroph/Lactotroph Adenoma Growth Hormone and Prolactin-Producing Adenoma A pituitary gland adenoma composed of acidophilic cells that produce both growth hormone and prolactin. It is characterized by the detection of growth hormone and prolactin within the same acidophilic cell, or an admixture of acidophilic and chromophobic cells that produce growth hormone and prolactin respectively. A non-malignant pituitary tumor that produces growth hormone and prolactin. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C131634 Platelet Disorder Platelet Abnormality A disorder of platelet function or platelet production that may cause increased bleeding. A disorder of platelet function or platelet production that may cause increased bleeding. C26323 Hematologic Disorder C90259 Pediatric Terminology C C3081 Heart Neoplasm Cardiac Neoplasm Cardiac Tumor|Neoplasm of Heart A benign or malignant neoplasm that affects the heart and/or the pericardium. Representative examples include atrial myxoma, cardiac myeloid sarcoma, and pericardial malignant mesothelioma. C3262 Neoplasm C90259 Pediatric Terminology C C3342 Lactotroph Adenoma Prolactinoma 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. A non-malignant prolactin-secreting pituitary tumor. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C45927 Mixed Somatotroph and Lactotroph Adenoma Mixed Somatolactotrope 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. A non-malignant pituitary tumor composed of both acidophilic and chromophobic cells that produce growth hormone and prolactin, respectively. C127194 Growth Hormone and Prolactin-Producing Adenoma C90259 Pediatric Terminology C C7461 Somatotroph Adenoma Growth Hormone-Producing Adenoma 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. A non-malignant pituitary tumor that produces growth hormone. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C7462 Corticotroph Adenoma ACTH-Producing Pituitary 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. A non-malignant pituitary neoplasm that produces adrenocorticotropic hormone. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C8011 Thyrotroph Adenoma Thyroid Stimulating Hormone-Producing Adenoma TSH-producing Adenoma A rare adenoma of the anterior lobe of the pituitary gland that produces thyrotropin. It is usually associated with goiter and hyperthyroidism. A benign pituitary tumor that produces thyroid-stimulating hormone. C3329 Pituitary Gland Adenoma C90259 Pediatric Terminology C C82339 Transient Abnormal Myelopoiesis Associated with Down Syndrome Transient Abnormal Myelopoiesis Transient Myeloproliferative Disorder A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission. A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients, the myeloid proliferation undergoes spontaneous remission. C104003 Congenital Hematological Disorder C90259 Pediatric Terminology