Prior to 2000 papillary thyroid carcinoma (PTC) was considered by most to be a sporadic disorder without familial predisposition. In contrast to this traditional teaching and as understood early on by Dr. Nadir Farid [1], approximately 5 percent of all PTC are familial. The evidence that supports this familial susceptibility is reviewed here and potential clinical implications are discussed. In addition, PTC may be a relatively infrequent component of other familial tumor syndromes. Although recent findings strongly support a familial PTC predisposition, the final proof will require the identification of the susceptibility genes. There is not yet convincing evidence to suggest that other nonmedullary thyroid carcinomas (follicular thyroid carcinoma, anaplastic thyroid carcinoma, and insular thyroid carcinoma) are familial.

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