Journal of Korean Thyroid Association  2012;5(2):124-131

doi:10.11106/jkta.2012.5.2.124

Multiple Endocrine Neoplasia and Familial Medullary Thyroid Carcinoma.

Young Sik CHOI 1

Affiliations

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Keywords

Multiple endocrine neoplasia; Familial medullary thyroid carcinoma

Country

Republic of Korea

Language

Korean

Abstract

Multiple endocrine neoplasia (MEN) is defined as a disorder with neoplasms in two or more different hormonal tissues in several members of a family. MEN1, or Wermer's syndrome, is inherited as an autosomal dominant trait. This syndrome is characterized by neoplasia of the parathyroid glands, enteropancreatic tumors, anterior pituitary adenomas, and other neuroendocrine tumors with variable penetrance. Inherited medullary thyroid carcinoma (MTC) consists of MEN2A, MEN2B, and familial medullary thyroid cancer (FMTC). The identification of hereditary MTC has been facilitated in recent years by direct analysis of germline RET proto-oncogene mutation.