An update on familial nonmedullary thyroid cancer

• Published in: Endocrine Vol. 68; no. 3; pp. 502 - 507
• Main Authors: Ammar, Sabrine A., Alobuia, Wilson M., Kebebew, Electron
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.06.2020; Springer Nature B.V
• Subjects: Diabetes; Endocrinology; Health risk assessment; Heredity; Humanities and Social Sciences; Internal Medicine; Medicine; Medicine & Public Health; Mini Review; multidisciplinary; Science; Thyroid cancer; Tumors; Nonsyndromic; Nonmedullary thyroid cancer; Syndromic; Papillary thyroid cancer; Familial
• ISSN: 1355-008X; 1559-0100
• DOI: 10.1007/s12020-020-02250-3

Familial nonmedullary thyroid cancer (FNMTC) constitutes 3–9% of all thyroid cancer cases. FNMTC is divided into two groups: syndromic and nonsyndromic. Nonsyndromic FNMTC is more common as compared with syndromic FNMTC. In syndromic FNMTC, patients are at risk of nonmedullary thyroid cancer (NMTC) and other tumors, and the susceptibility genes are known. In nonsyndromic FNMTC, NMTC is the major feature of the disease and occurs in isolation with an autosomal dominant pattern of inheritance and variable penetrance. New data have emerged on the genetics, clinical characteristics, and outcomes of patients with FNMTC that may have clinical relevance in the management of patients. In this review, we focus on newly characterized syndromic FNMTC entities, criteria for screening and surveillance of nonsyndromic FNMTC, and the classification of nonsyndromic FNMTC as well as the genetic background and heterogeneity of nonsyndromic FNMTC.