Abstract
Approximately 3%–9% of all nonmedullary thyroid cancers are inherited. Familial nonmedullary thyroid cancer (FNMTC) is classified syndromic (part of a familial cancer syndrome) or as nonsyndromic (the predominant or only feature being nonmedullary thyroid cancer). Nonsyndromic FNMTC account for 95% of FNMTC cases is more aggressive than sporadic nonmedullary thyroid cancer and has a younger age at presentation when three or more first-degree relatives are affected. Although the risk of nonmedullary thyroid cancer is higher than in the general population in syndromic FNMTC, it is commonly a minor component or an infrequent manifestation of the syndrome. Syndromic FNMTC includes familial adenomatous polyposis, PTEN hamartoma tumor syndrome, Carney's complex type 1, Werner, Pendred and DICER1 syndromes, and ataxia-telangiectasia. In this chapter, we review the genetics of syndromic and nonsyndromic FNMTC and the clinicopathologic features of syndromic and nonsyndromic FNMTC.
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