Thyroid cancer of follicular cell origin in inherited tumor syndromes.

[pendred syndrome]

Well-differentiated thyroid cancer accounts for 95 % of thyroid malignancies . In contrast to medullary thyroid carcinoma , in which about 25 % are familial , only 5 % of follicular cell-derived thyroid carcinomas are a component of a familial cancer syndrome . The familial follicular cell-derived tumors or nonmedullary thyroid carcinoma encompass a heterogeneous group of diseases , and are classified into 2 distinct groups : syndromic-associated tumors , occurring in syndromes in which nonmedullary thyroid carcinomas are the predominant tumor encountered , and nonsyndromic tumors , those occurring in tumor syndromes in which thyroid involvement is a minor component . The first group , syndromic-associated tumors , includes phosphase and tensin (PTEN )-hamartoma tumor syndrome /Cowden syndrome , familial adenomatous polyposis /Gardner syndrome , Carney complex type 1 , Werner syndrome , and Pendred syndrome . Other syndromes , as McCune Albright syndrome , Peutz-Jeghers syndrome , and Ataxia -teleangiectasia syndrome may be associated with the development of follicular cell-derived tumors , but the link is less established than the above syndromes . The syndromic-associated tumors are the focus of this review . The second group of familial follicular cell-derived tumors syndromes or nonsyndromic tumors , in which nonmedullary thyroid carcinomas are the major findings , include pure familial papillary thyroid carcinoma , with or without oxyphilia , familial papillary thyroid carcinoma with papillary renal cell carcinoma , and familial papillary thyroid carcinoma with multinodular goiter . This review will discuss the clinical and pathological findings of the patients with familial syndrome-associated tumors : PTEN -hamartoma tumor syndrome /Cowden syndrome , familial adenomatous polyposis syndrome , Carney complex type 1 , Werner syndrome , and Pendred syndrome .

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pendred syndrome

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