J Korean Thyroid Assoc.  2012 Nov;5(2):124-131. 10.11106/jkta.2012.5.2.124.

Multiple Endocrine Neoplasia and Familial Medullary Thyroid Carcinoma

• Choi YS

• KMID: 2095101
• DOI: http://doi.org/10.11106/jkta.2012.5.2.124

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.

Keyword

Multiple endocrine neoplasia; Familial medullary thyroid carcinoma

MeSH Terms

Carcinoma, Medullary
Humans
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Multiple Endocrine Neoplasia Type 2a
Multiple Endocrine Neoplasia Type 2b
Neuroendocrine Tumors
Parathyroid Glands
Penetrance
Pituitary Neoplasms
Proto-Oncogenes
Thyroid Gland
Thyroid Neoplasms
Carcinoma, Medullary
Multiple Endocrine Neoplasia Type 2a
Thyroid Neoplasms