Current Guidelines for Management of Medullary Thyroid Carcinoma

Endocrinol Metab (Seoul). 2021 Jun;36(3):514-524. doi: 10.3803/EnM.2021.1082. Epub 2021 Jun 22.


Medullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor originating from the parafollicular cells. The diagnostic and therapeutic strategies for the condition are different from those used for well-differentiated thyroid cancer. Since the 2015 American Thyroid Association guidelines for the diagnosis and treatment of MTC, the latest, including the National Comprehensive Cancer Network and European Association for Medical Oncology guidelines have been updated to reflect several recent advances in the management of MTC. Advances in molecular diagnosis and postoperative risk stratification systems have led to individualized treatment and follow-up strategies. Multi-kinase inhibitors, such as vandetanib and cabozantinib, can prolong disease progression-free survival with favorable adverse effects. In addition, potent selective rearranged during transfection (RET) inhibitors (selpercatinib and pralsetinib) have shown a promising efficacy in recent clinical trials. This review summarizes the management of MTC in recent guidelines focused on sporadic MTC.

Keywords: Cabozantinib; Guidelines; Pralsetinib; Precision medicine; Selpercatinib; Thyroid cancer, medullary.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Carcinoma, Neuroendocrine* / diagnosis
  • Carcinoma, Neuroendocrine* / drug therapy
  • Humans
  • Protein Kinase Inhibitors / therapeutic use
  • Thyroid Neoplasms* / diagnosis
  • Thyroid Neoplasms* / drug therapy


  • Protein Kinase Inhibitors