Familial nonmedullary thyroid carcinoma

It is well-known that medullary thyroid carcinoma occurs in a familial form as part of the multiple endocrine neoplasia (MEN) 2 syndromes. However, it is less well-recognized that nonmedullary thyroid carcinoma (NMTC) sometimes is familial. Arising from the thyroid epithelial cell, the NMTCs include...

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Bibliographic Details
Published inSeminars in surgical oncology Vol. 16; no. 1; p. 16
Main Authors Malchoff, C D, Malchoff, D M
Format Journal Article
LanguageEnglish
Published United States 01.01.1999
Subjects
Adenocarcinoma, Follicular - genetics
Adenocarcinoma, Follicular - pathology
Adult
Carcinoma - genetics
Carcinoma - pathology
Carcinoma, Papillary - genetics
Carcinoma, Papillary - pathology
Humans
Thyroid Neoplasms - genetics
Thyroid Neoplasms - pathology
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Summary:It is well-known that medullary thyroid carcinoma occurs in a familial form as part of the multiple endocrine neoplasia (MEN) 2 syndromes. However, it is less well-recognized that nonmedullary thyroid carcinoma (NMTC) sometimes is familial. Arising from the thyroid epithelial cell, the NMTCs include papillary, follicular, and anaplastic thyroid carcinoma. Although most NMTC are sporadic, there is increasing evidence for a familial form. When inherited, NMTC is autosomal dominant with partial penetrance, and it is not associated consistently with other malignancies. The average age of onset is about 38 years, and in some cases, it may be more aggressive than sporadic PTC; up to 5% of subjects with NMTC have a family history positive for the same disorder. The etiologic gene(s) have not been identified, although positional cloning of these genes may be possible. The evidence for and characteristics of familial NMTC will be reviewed, and the clinical and research implications will be discussed.
ISSN:8756-0437
DOI:10.1002/(SICI)1098-2388(199901/02)16:1<16::AID-SSU4>3.0.CO;2-7