Familial thyroid carcinoma: the road less traveled in thyroid pathology – an update

This paper reviews the pathologic and clinicopathologic aspects of thyroid cancers and neoplasms which occur in familial settings. The data and details of C cell lesions are reviewed and a brief overview of the molecular-morphological correlations is presented. Familial tumors of follicular derivation are also described with a review of the many syndromes associated with these lesions. These tumors which may be papillary, follicular or Hurthle cell can occur solely as thyroid neoplasms without extrathyroidal manifestations or may accompany syndromes in which the extrathyroidal lesions are of primary prognostic importance (e.g. familial adenomatous polyposis). The pathologist may play a critical role in the identification of these syndromes by recognizing particular patterns of thyroid tumors (e.g. cribriform morular variant of papillary carcinoma). These issues are reviewed in this article.