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Familial Non-medullary Thyroid Carcinoma Syndrome (FNMTC) and Familial Syndromes Associated with Thyroid Cancer

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Abstract

Familial non-medullary thyroid cancer (FNMTC) constitutes 3–9% of all thyroid cancer (TC) cases and is divided into syndromic and non-syndromic FNMTC. In syndromic FNMTC, patients are at risk of non-medullary thyroid cancer and multiple other tumors with syndrome-specific clinical features, and the susceptibility genes are known. In non-syndromic FNMTC, non-medullary thyroid cancer is the major feature of the disease. New data have emerged on the genetics, clinical characteristics, and outcomes of patients with FNMTC. This chapter will summarize emerging data regarding classifications and definitions of FNMTC, criteria for screening and surveillance in patients with FNMTC, evaluation of the extent of disease, candidate susceptibility genes, and pattern of inheritance in FNMTC. The aim is to provide guidance for managing patients with FNMTC.

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Authors and Affiliations

  1. Surgical Oncology Program, Endocrine Surgery Section, National Cancer Institute, NIH, Bethesda, MD, USA

    Samira Mercedes Sadowski

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  1. Samira Mercedes Sadowski
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Correspondence to Samira Mercedes Sadowski .

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Editors and Affiliations

  1. Department of Surgery, Jersey Shore University Medical Center, Neptune, NJ, USA

    Alexander L. Shifrin

  2. Division of Endocrine and Metabolic Surgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy

    Marco Raffaelli

  3. The Claire and John Bertucci Endowed Chair in Thyroid Surgical Oncology, Harvard Medical School, Boston, MA, USA

    Gregory W. Randolph

  4. Department of Surgery, Linköping University, Linköping, Sweden

    Oliver Gimm

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Sadowski, S.M. (2021). Familial Non-medullary Thyroid Carcinoma Syndrome (FNMTC) and Familial Syndromes Associated with Thyroid Cancer. In: Shifrin, A.L., Raffaelli, M., Randolph, G.W., Gimm, O. (eds) Endocrine Surgery Comprehensive Board Exam Guide. Springer, Cham. https://doi.org/10.1007/978-3-030-84737-1_32

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