• Katerina Green, Justin Hintze, and James Paul O'Neill.
• Royal College of Surgeons in Ireland, Dublin, Ireland. katerinagreen@rcsi.ie.
• Ir J Med Sci. 2022 Dec 1; 191 (6): 246124662461-2466.

AbstractMedullary thyroid cancer (MTC) accounts for only 4% of thyroid carcinomas but 15% of thyroid cancer deaths. MTC is a tumour of the calcitonin secreting parafollicular C cells in the thyroid which can occur sporadically or be hereditary in multiple endocrine neoplasias type 2 syndromes due to germline RET mutations. Sporadic forms of MTC can also be caused by mutations in the RET protooncogene. MTC commonly presents in a late stage, with 70% of patients presenting with local nodal metastasis. Currently, the only curative treatment for MTC is surgical removal. The aim of this paper is to describe the current guidelines and progressions of the surgical management of MTC and to highlight up-and-coming chemotherapies. A database literature review was completed utilizing PubMed to cumulate the extant literature, screening for most recent guidelines and publications regarding the management of MTC. Current guidelines were described by the American Thyroid Association in 2015. Controversial publications continue to present supporting evidence for varying degrees of thyroidectomy and neck dissections. Recently, researchers have been exploring non-surgical options including external beam radiotherapy and multikinase inhibitors such as vandetanib and cabozantinib for the treatment of MTC. Surgical management of MTC remains controversial and varies significantly dependent on the extent of disease. Chemotherapeutic options have undetermined effects on survival to date.© 2021. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.

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