Medullary thyroid cancer is a tumor arising from the parafollicular cells, or C cells, of the thyroid gland. Medullary thyroid cancer produces calcitonin, and elevated calcitonin level is an essential feature of this tumor. It is derived from the medullary C cells that are of neural crest origin. Recent advances in molecular pathogenesis and genetic testing have led to risk stratification of the patients and the identification of molecular targets for therapy. Prophylactic thyroidectomy is recommended for patients with mutations that put them at high risk. Various tyrosine kinase inhibitors are approved for use in progressive, metastatic medullary thyroid cancer.
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