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

Surg Oncol Clin N Am. 2023 Apr;32(2):233-250. doi: 10.1016/j.soc.2022.10.002.

Abstract

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.

Keywords: Lymph node dissection; Medullary thyroid cancer; Medullary thyroid carcinoma; Neuroendocrine tumor; RET; Targeted therapy; Thyroidectomy.

Publication types

  • Review

MeSH terms

  • Carcinoma, Medullary* / genetics
  • Carcinoma, Medullary* / pathology
  • Carcinoma, Medullary* / surgery
  • Carcinoma, Neuroendocrine* / drug therapy
  • Carcinoma, Neuroendocrine* / genetics
  • Humans
  • Proto-Oncogene Mas
  • Thyroid Neoplasms* / drug therapy
  • Thyroid Neoplasms* / genetics

Substances

  • Proto-Oncogene Mas

Supplementary concepts

  • Thyroid cancer, medullary