Management of Medullary Thyroid Cancer

Endocrinol Metab Clin North Am. 2019 Mar;48(1):285-301. doi: 10.1016/j.ecl.2018.11.006. Epub 2018 Dec 26.

Abstract

Medullary thyroid cancer (MTC) is rare but aggressive. It can be cured only if intrathyroid at diagnosis. MTC can be sporadic (75%) or familial (25%) and the 2 forms are distinguished by RET mutations analysis. Calcitonin is the specific serum marker; its doubling time is the most important prognostic factor for survival and progression; 30% of MTC patients have distant metastases at diagnosis and, when progressing, systemic therapy with vandetanib or cabozantinib should be considered. Before starting this treatment, the possibility of using a local treatment should be evaluated to delay systemic therapy. A multidisciplinary team should care for these patients.

Keywords: CEA; Calcitonin; MEN; Medullary thyroid cancer; RET.

Publication types

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

MeSH terms

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

Substances

  • Protein Kinase Inhibitors
  • Calcitonin

Supplementary concepts

  • Thyroid cancer, medullary