Abstract
The diagnosis and treatment of medullary thyroid cancer (MTC) has changed considerably over the past 40 years, although survival has not improved over the past three decades. From the first clinical descriptions of MTC and the multiple endocrine neoplasia syndromes to the identification of the RET proto-oncogene, the concept of prophylactic thyroidectomy has become a reality for some families. The surgery is usually performed by high-volume surgeons in specialized centers with results that rarely leave patients with complications but with maximum survival benefit. For patients with unresectable or metastatic disease, many new targeted agents have been developed, some of which have shown great promise and are close to being approved for a disease which hitherto has had no systemic treatment options. This article highlights the important advances in the diagnosis and management of MTC.