Management of hereditary medullary thyroid carcinoma

Endocrine
Theodora Pappa, Maria Alevizaki

Abstract

Hereditary medullary thyroid carcinoma (MTC) represents up to one-third of MTC cases and includes multiple endocrine neoplasia syndrome type 2A (and its variant familial MTC) and 2B. The aim of this paper is to provide an overview of the disease focusing on the management of hereditary MTC patients, who have already developed tumor, as well as discuss the recommended approach for asymptomatic family members carrying the same mutation. A PubMed search was performed to review recent literature on diagnosis, genetic testing, and surgical and medical management of hereditary MTC. The wide use of genetic testing for RET mutations has markedly influenced the course of hereditary MTC. Prophylactic thyroidectomy of RET carriers at an early age eliminates the risk of developing MTC later in life. Pre-operative staging is a strong prognostic factor in patients, who have developed MTC. The use of recently approved tyrosine kinase inhibitors (vandetanib, cabozantinib) holds promising results for the treatment of unresectable, locally advanced, and progressive metastatic MTC. Genetic testing of the RET gene is a powerful tool in the diagnosis and prognosis of MTC. Ongoing research is expected to add novel treatment options for patients with...Continue Reading

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Citations

Dec 13, 2016·British Medical Bulletin·Anju Kulkarni, Helena Carley
Aug 23, 2017·International Journal of Molecular Sciences·Dagmara RusinekBarbara Jarzab
Jul 26, 2019·Journal of Pediatric Endocrinology & Metabolism : JPEM·Cristina Garcés VisierJose Lorenzo Alonso Calderón
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Sep 2, 2018·Pediatrics in Review·Sarah K AnisowiczAnita M Pedersen
Apr 6, 2021·Journal of Pediatric Surgery·Javier OrdóñezJuan Carlos de Agustín

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