ABSTRACT
This chapter provides a comprehensive review of Medullary thyroid cancer (MTC), including its clinical presentation and the different behaviors of hereditary versus sporadic disease, initial surgical management, prognostic indicators, and the systemic treatments now available as a standard of care for patients with advanced and progressive MTC. MTC, a neuroendocrine malignancy of thyroid C cells, accounts for approximately 1–2% of all thyroid malignancies. Since 1993, strong correlations observed between RET mutations and their respective clinical expression drove consensus recommendations for evaluation, treatment to prevent morbidity and mortality associated with hereditary MTC. Patients diagnosed with MTC should be evaluated for symptoms of dysphagia, dyspnea, and hoarseness, which may indicate the presence of locally advanced disease. Bone metastases in MTC are often clinically silent. In a retrospective study of over 1000 patients with MTC from a single center, 25% of patients with bone metastases were identified within 3 months of MTC diagnosis.
