Abstract
Medullary thyroid cancer (MTC) accounts for 5-10% of thyroid cancer. The majority, 75-80%, of MTC are sporadic with the remainder being hereditary secondary to a mutation in the RET proto-oncogene. Hereditary MTC may be isolated as in familial medullary thyroid cancer or associated with multiple endocrine neoplasia syndrome types 2A and 2B. The primary treatment modality for sporadic MTC is total thyroidectomy and central compartment neck dissection; consideration of lateral neck dissection should be based on preoperative imaging findings. The timing of prophylactic thyroidectomy is dependent on the specific RET codon mutation.