Which familial syndromes increase the risk for papillary thyroid carcinoma (PTC)?
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Which familial syndromes increase the risk for papillary thyroid carcinoma (PTC)?
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@gavrie Unlike medullary thyroid carcinoma, papillary thyroid cancer is not a part of multiple endocrine neoplasia syndromes. However, uncommon familial syndromes such as familial adenomatous polyposis (FAP), Gardner syndrome and Cowden disease may be associated with thyroid papillary tumors in about 5% of cases.
In particular, FAP is associated with elevated risk of the rare cribriform-morula variant of papillary thyroid carcinoma (CMV-PTC). A study of 129 Japanese patients with FAP who underwent screening with neck ultrasonography found 11 cases of papillary thyroid cancer, eight of which were CMV-PTC. All the patients with CMV-PTC were women 35 years of age or younger.