Current Guidelines for Management of Medullary Thyroid Carcinoma

Abstract

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.

Fig. 1

Postoperative management in patients with medullary thyroid cancer (MTC) according to dynamic risk stratification in European Association for Medical Oncology (ESMO) guidelines. CEA, carcinoembryonic antigen; US, ultrasonography. ^aAmerican Thyroid Association (ATA) guidelines recommend that serum calcitonin and CEA should be measured after 3 months postoperatively; ^bOther imaging modalities including contrast-enhanced (CE) computed tomography (CT) of neck, chest, abdomen with liver protocol, and bone scan, axial magnetic resonance imaging (MRI) depending on the tumor stage, serum calcitonin (≥150 pg/mL) and CEA; ^cNegative other imaging modalities, consider [¹⁸F]-fluoro-2-deoxy-D-glucose (F18-FDG) positron emission tomography-CT or Gallium-68 DOTATATE or CE MRI with neck, chest, abdomen with liver protocol in National Comprehensive Cancer Network (NCCN) guidelines; ^dStable or progressive disease according to Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1.