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Multiple Endocrine Neoplasia Type 2

Abstract

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NORD is very grateful to Maria Luisa Brandi, MD, PhD, Director, Regional Center for Hereditary Endocrine Tumors, University of Florence, Florence, Italy, for assistance in the preparation of this report.

Synonyms of Multiple Endocrine Neoplasia Type 2

  • MEN-2
  • MEN-2 syndrome

Disorder Subdivisions

  • familial medullary thyroid carcinoma (FMTC)
  • MEN 2A (Sipple syndrome)
  • MEN 2B (multiple neuroma syndrome)
  • multiple endocrine neoplasia type 2A
  • multiple endocrine neoplasia type 2B
  • Wagenmann-Froboese syndrome

General Discussion

Multiple endocrine neoplasia (MEN) type 2 is a rare genetic disorder characterized by an increased risk of developing a specific form of thyroid cancer (medullary thyroid carcinoma) and benign tumors affecting additional glands of the endocrine system. The endocrine system is the network of glands that secrete hormones into the bloodstream where they travel to various areas of the body. These hormones regulate the chemical processes (metabolism) that influence the function of various organs and activities within the body. Hormones are involved in numerous vital processes including regulating heart rate, body temperature and blood pressure. Glands affected in MEN type 2 may secrete excessive amounts of hormones into the bloodstream, which can result in a variety of symptoms.

MEN type 2 is divided into three subtypes: MEN type 2A, MEN type 2B, and familial medullary thyroid carcinoma (FMTC). Medullary thyroid carcinoma (MTC) affects nearly all individuals with MEN type 2 regardless of the subtype. Additional symptoms may occur depending upon the specific subtype present. In FMTC, no other symptoms besides the carcinoma are present. MEN type 2 can run in families or can occur as the result of a spontaneous genetic change (i.e., new mutation) that occurs randomly for no apparent reason.

Organizations related to Multiple Endocrine Neoplasia Type 2

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

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