Medullary thyroid cancer (MTC) is a malignant tumor of the parafollicular C cells of the thyroid and comprises only 1-2% of all thyroid cancer cases. Unlike most differentiated thyroid cancer, MTC is associated with a mean survival of 8.6 years and accounts for a disproportionate 8.6% of thyroid cancer deaths. Surgery is the mainstay of treatment for loco-regional disease and the only current means of cure for MTC. The relatively low incidence of MTC has made the comprehensive study of this disease difficult and most research to date has been based largely on single institution, retrospective, and/or non-randomized studies. Despite various professional organizations such as the American Thyroid Association establishing guidelines for the diagnosis and treatment of patients with MTC, there is still significant variation in actual practice patterns with regard to the extent of surgery, as well as the management of persistent or recurrent disease. The purpose of this review is to discuss the latest updates in the surgical treatment of MTC, as well as the management of locally advanced, recurrent, and metastatic disease based on the most recent data and expert consensus guidelines.
Keywords: Endocrine tumor; Lymphadenectomy; Medullary thyroid cancer; Surgery; Thyroidectomy.