A Contemporary Review of the Treatment of Medullary Thyroid Carcinoma in the Era of New Drug Therapies

• Published in: Surgical oncology clinics of North America Vol. 32; no. 2; pp. 233 - 250
• Main Authors: Seib, Carolyn D., Beck, Thomas C., Kebebew, Electron
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-04-2023
• Subjects: Carcinoma, Medullary - genetics; Carcinoma, Medullary - pathology; Carcinoma, Medullary - surgery; Carcinoma, Neuroendocrine - drug therapy; Carcinoma, Neuroendocrine - genetics; Humans; Lymph node dissection; Medullary thyroid cancer; Medullary thyroid carcinoma; Neuroendocrine tumor; Proto-Oncogene Mas; RET; Targeted therapy; Thyroid Neoplasms - drug therapy; Thyroid Neoplasms - genetics; Thyroidectomy; RET; Lymph node dissection; Targeted therapy; Neuroendocrine tumor; Thyroidectomy; Medullary thyroid carcinoma; Medullary thyroid cancer
• ISSN: 1055-3207; 1558-5042
• DOI: 10.1016/j.soc.2022.10.002

Medullary thyroid cancer (MTC) is a rare neuroendocrine tumor that can be sporadic or inherited and is often associated with mutations in the RET (Rearranged during Transfection) oncogene. The primary treatment for MTC is surgical resection of all suspected disease, but recent advances in targeted therapies for MTC, including the selective RET inhibitors selpercatinib and pralsetinib, have led to changes in the management of patients with locally advanced, metastatic, or recurrent MTC. In this article, we review updates on the evaluation and management of patients with MTC, focusing on new and emerging therapies that are likely to improve patient outcomes.