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Multiple endocrine neoplasia type 2 (also known as "Pheochromocytoma (codons 630 and 634) and amyloid producing medullary thyroid carcinoma", [1] "PTC syndrome," [1] and "Sipple syndrome" [1]) is a group of medical disorders associated with tumors of the endocrine system.
Medullary thyroid cancer is a form of thyroid carcinoma which originates from the parafollicular cells (C cells), which produce the hormone calcitonin. [1] Medullary tumors are the third most common of all thyroid cancers and together make up about 3% of all thyroid cancer cases. [ 2 ]
This variant can cause medullary thyroid cancer and pheochromocytoma. Presentation can include a Marfanoid body, enlarged lips, and ganglioneuromas. [citation needed] MEN 2B typically manifests before a child is 10 years old. Affected individuals tend to be tall and lanky, with an elongated face and protruding, blubbery lips.
The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries. It is currently in its third revision (ICD-O-3). ICD-10 includes a list of ...
Thyroid neoplasm is a neoplasm or tumor of the thyroid. It can be a benign tumor such as thyroid adenoma, [1] or it can be a malignant neoplasm (thyroid cancer), such as papillary, follicular, medullary or anaplastic thyroid cancer. [2] Most patients are 25 to 65 years of age when first diagnosed; women are more affected than men.
FMTC = familial medullary thyroid cancer Micrograph of a medullary thyroid carcinoma, as may be seen in MEN 2A and MEN 2B. H&E stain. MEN 2B is sometimes known as MEN 3 and the designation varies by institution (c.f. www.ClinicalReview.com). Although a variety of additional eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome ...
Follicular thyroid cancer (10 to 20% of cases [35]) – occasionally seen in people with Cowden syndrome. Some include Hürthle cell carcinoma as a variant and others list it as a separate type. [4] [37] Medullary thyroid cancer (5 [35] to 8% of cases) – cancer of the parafollicular cells, often part of multiple endocrine neoplasia type 2. [38]
NETs include certain tumors of the gastrointestinal tract and of the pancreatic islet cells, [1] certain thymus and lung tumors, and medullary carcinoma of the parafollicular cells of the thyroid. [1] Tumors with similar cellular characteristics in the pituitary, parathyroid, and adrenomedullary glands are sometimes included [9] or excluded. [1]