Medullary thyroid cancer: an update of new guidelines and recent developments.

PURPOSE OF REVIEW: Medullary thyroid cancer (MTC) is an uncommon malignancy. Its low incidence has limited both widespread clinical expertise and definitive large randomized clinical trials. Variation in practice patterns exist in the United States with regard to diagnosis, treatment, and long-term management. We review the most recent guidelines on management of this challenging neuroendocrine malignancy.

RECENT FINDINGS: Newly identified re-arranged during transfection point mutations have added to clinicians' disease prognostic accuracy, which have been incorporated in the new MTC treatment guidelines. The study of tumor marker doubling times has guided the extent of surgery and lymphadenectomy for MTC. Although data are limited, standard chemotherapy and radiation therapy have not been shown to be effective in the treatment of MTC. Newer targeted drug therapies are promising and are being examined in therapeutic clinical trials.

SUMMARY: There have been several recent advances in the molecular biology, diagnosis, imaging, and treatment options for MTC. Downstaging and treating metastatic disease more effectively may improve overall survival of MTC patients. Dissemination of standardized guidelines is important for optimal treatment with less variation in quality of care.