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Medullary Thyroid Cancer

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Abstract

Medullary thyroid carcinoma (MTC) is an uncommon neuroendocrine tumor (NET) derived from the parafollicular C cells of the thyroid gland. Calcitonin secretion is a universal feature of the tumor. Approximately 25–30 % of the patients present with autosomal dominant hereditary forms as part of multiple endocrine neoplasia (MEN)—MEN-2A, MEN-2B, or familial MTC syndromes. RET proto-oncogene mutations are responsible for the pathogenesis. Hereditary forms are secondary to germline mutations in the RET gene with different penetration rates causing a diversity of disease phenotypes. Majority of the sporadic cases harbor somatic RET gene mutations, whereas about 5 % of those display germline RET gene mutations. Identification of patients genetically at high risk is critical, because affected individuals are best treated with early and prophylactic surgery. Surgical resection remains the principal treatment modality with total thyroidectomy and extended cervical lymph node dissection. Impact of radiotherapy and chemotherapy in the management of MTC is limited, and prognosis of progressive and metastatic disease remains dismal. Improved comprehension of the molecular alterations and discovery of pathways involved in the pathogenesis of MTC have led to the development of specific targeted inhibitors against tyrosine kinases that have changed the way metastatic disease is treated. Vandetanib and cabozantinib are approved by the FDA in the treatment of advanced MTC. Although much progress has been made, there exists an apparent “unmet medical need” for innovative therapeutic approaches in advanced MTC.

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Authors and Affiliations

  1. Department of Medical Oncology, Gazi University Faculty of Medicine, Besevler, Ankara, 06500, Turkey

    Aydin Ciltas, Yusuf Gunaydin & Mustafa Benekli MD

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  1. Aydin Ciltas
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  2. Yusuf Gunaydin
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Correspondence to Mustafa Benekli MD .

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  1. Department of Medical Oncology, Hacettepe University Cancer Institute, Ankara, Turkey

    Suayib Yalcin

  2. Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden

    Kjell Öberg

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Ciltas, A., Gunaydin, Y., Benekli, M. (2015). Medullary Thyroid Cancer. In: Yalcin, S., Öberg, K. (eds) Neuroendocrine Tumours. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45215-8_22

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