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History and Epidemiology

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Medullary Thyroid Cancer

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Abstract

Medullary thyroid carcinoma (MTC) was recognized as a distinct pathologic type of thyroid cancer relatively recently. A sequence of important discoveries led to the description of MTC, including the identification of the separate population of thyroid parafollicular or C-cells which derive embryologically from the neural crest, and the discovery that this cell population was responsible for the production of the peptide hormone calcitonin. The unique features of MTC include its familial associations, intermediate level of biologic aggressiveness, and its lack of susceptibility to radioiodine making complete surgical removal of all cancer and lymphatic metastases the primary treatment goal. Germline mutations in the RET protooncogene are responsible for the endocrine tumors that develop in association with the multiple endocrine neoplasia type 2 (MEN2) syndromes, and somatic mutations in the same gene underlie a significant proportion of sporadic tumors. Importantly, direct genetic testing allows for presymptomatic identification of patients at high risk for developing thyroid cancer during their lifetime. Early thyroidectomy may be performed in patients who are found to have inherited a MEN2 disease-associated mutation, with the goal of removing the end organ at risk before invasive malignancy develops. Prophylactic thyroidectomy for patients with MEN2 was one of the first, and is perhaps still one of the best, examples of a surgical intervention based on genetic testing that is intended to completely prevent subsequent cancer development in patients with an inherited cancer susceptibility.

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Authors and Affiliations

  1. Baylor Scott and White Health, Temple, TX, USA

    Mary M. Mrdutt

  2. Division of Surgical Oncology, Department of Surgery, Baylor Scott & White Health, Texas A&M University Health Science Center, MS-01-730C, 2401 S. 31st Street, Temple, TX, 76508, USA

    Terry C. Lairmore

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Correspondence to Terry C. Lairmore .

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Editors and Affiliations

  1. Associate Professor of Surgery; Chief, Section of Endocrine Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Tracy S. Wang

  2. Donald C. Ausman Family Foundation Professor in Surgery and Chair, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Douglas B. Evans

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Mrdutt, M.M., Lairmore, T.C. (2016). History and Epidemiology. In: Wang, T., Evans, D. (eds) Medullary Thyroid Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-39412-1_1

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