Multiple endocrine neoplasia type 2
Multiple endocrine neoplasia, type 2 (MEN2) is an inherited disorder in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:
- Adrenal (about half the time)
- Parathyroid (20% of the time)
- Thyroid (almost all of the time)
MEN2 is caused by a defect in the RET gene. This defect causes many tumors to appear in the same person, but not necessarily at the same time. The adrenal tumor is called a pheochromocytoma, and the thyroid tumor is a medullary carcinoma of the thyroid. The condition is usually inherited in an autosomal dominant pattern.
MEN2 is divided into three subtypes: type 2A, type 2B, and familial medullary thyroid carcinoma (FMTC). These subtypes differ in their characteristic signs and symptoms and risk of specific tumors. The features of this disorder are relatively consistent within any one family.
- Dugdale DC, Chen YB. Multiple endocrine neoplasia (MEN) II. MedlinePlus. 2010; http://www.nlm.nih.gov/medlineplus/ency/article/000399.htm. Accessed 3/18/2011.
- Multiple endocrine neoplasia. Genetics Home Reference (GHR). 2006; http://ghr.nlm.nih.gov/condition/multiple-endocrine-neoplasia. Accessed 8/25/2010.
- Genetics Home Reference (GHR) contains information on Multiple endocrine neoplasia type 2. This website is maintained by the National Library of Medicine.
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- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
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