Multiple Endocrine Neoplasia and Familial Medullary Thyroid Carcinoma.
10.11106/jkta.2012.5.2.124
- Author:
Young Sik CHOI
1
Author Information
1. Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea. yschoi@kosinmed.or.kr
- Publication Type:Review
- Keywords:
Multiple endocrine neoplasia;
Familial medullary thyroid carcinoma
- MeSH:
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
- From:Journal of Korean Thyroid Association
2012;5(2):124-131
- CountryRepublic 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.
