[Multiple endocrine neoplasia].
Multiple endocrine neoplasia (MEN) is dominantly inherited tumor syndromes characterized by multiple endocrine hyperfunctions due to hyperplastic or adenomatous as well as carcinomatous abnormalities. The syndromes are classified into MEN 1, 2A and 2B according with the complex of abnormalities. Hyperparathyroidism is the most common manifestation of MEN 1 and is also associated with MEN 2A. Recent genetic studies revealed that alleleic deletions on chromosome 11q13 are involved in the monoclonal development of endocrine cell in MEN 1 and germ-line mutations of RET proto-oncogene are associated with MEN 2A and 2B. This paper reviews clinical characteristics of MEN 1, 2A and 2B in Japan and presents the respective case reports including the gene analysis.
Morimoto
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D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000235:genetics*; D018813:Multiple Endocrine Neoplasia Type 2a / Q000150:complications / Q000235:genetics*; D018814:Multiple Endocrine Neoplasia Type 2b / Q000150:complications / Q000235:genetics*; D009154:Mutation; D000090063:Proto-Oncogene Mas; D011519:Proto-Oncogenes