The Impact of Family History on Non-Medullary Thyroid Cancer

• Published in: European journal of surgical oncology Vol. 42; no. 10; pp. 1455 - 1463
• Main Authors: Nixon, Iain J., MD, PhD, Suárez, Carlos, MD, PhD, Simo, Ricard, MD, MBChB, FRCS, Sanabria, Alvaro, MD, MSc, PhD, Angelos, Peter, MD, Rinaldo, Alessandra, MD, FRCSEd ad hominem, FRCS (Eng, Ir) ad eundem, FRCSGlasg, FACS, Rodrigo, Juan P., MD, PhD, Kowalski, Luiz P., MD, PhD, Hartl, Dana M., MD, PhD, Hinni, Michael L., MD, Shah, Jatin P., MD, PhD (Hon), MS, FACS, FRCSEd (Hon), FRACS (Hon), FDSRCS, Ferlito, Alfio, MD, DLO, DPath, FRCSEd ad hominem, FRCS (Eng, Glasg, Ir) ad eundem, FDSRCS ad eundem, FACS, FHKCORL, FRCPath, FASCP, IFCAP
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.10.2016
• Subjects: Familial thyroid cancer; Family history; Hematology, Oncology and Palliative Medicine; Humans; Lymphatic Metastasis; Prognosis; Surgery; Thyroid cancer; Thyroid Neoplasms - genetics; Thyroid Neoplasms - pathology; Thyroid Neoplasms - therapy; Thyroidectomy; familial thyroid cancer; family history; Thyroid cancer; Familial thyroid cancer; Family history
• ISSN: 0748-7983; 1532-2157
• DOI: 10.1016/j.ejso.2016.08.006

Abstract Introduction Around 10% of patients with non-medullary thyroid cancer (NMTC) will have a positive family history for the disease. Although many will be sporadic, families where 3 first-degree relatives are affected can be considered to represent true familial non-medullary thyroid cancer (FNMTC). The genetic basis, impact on clinical and pathological features, and overall effect on prognosis are poorly understood. Methods A literature review identified articles which report on genetic, clinical, therapeutic and screening aspects of FNMTC. The results are presented to allow an understanding of the genetic basis and the impact on clincal-pathological features and prognosis in order to inform clinical decision making. Results The genetic basis of FNMTC is unknown. Despite this, significant progress has been made in identifying potential susceptibility genes. The lack of a test for FNMTC has led to a clinical definition requiring a minimum of 3 first-degree relatives to be diagnosed with NMTC. Although some have shown an association with multi-centric disease, younger age and increased rates of extra-thyroidal extension and nodal metastases, these findings are not supported by all. The impact of FNMTC is unclear with all groups reporting good outcome, and some finding an association with more aggressive disease. The role of screening remains controversial. Conclusion FNMTC is rare but can be diagnosed clinically. Its impact on prognostic factors and the subsequent role in influencing management is debated. For those patients who present with otherwise low-risk differentiated thyroid cancer, FNMTC should be included in risk assessment when discussing therapeutic options.