Abstract

Medullary thyroid cancer (MTC) is a neuroendocrine malignancy of the calcitonin producing thyroid C cells, accounting for 5–10% of all thyroid cancers. While the majority of cases are sporadic (75%), hereditary syndromes account for 25% of all cases and should be considered whenever evaluating a patient. Compared to differentiated thyroid cancer, MTC is more aggressive yet remains a relatively indolent malignancy, with 10-year survival rates reported from 69% to 89%. Sporadic cases usually present with a palpable thyroid mass. Hereditary cases may be detected and treated on the basis of a genomic diagnosis once the syndrome is diagnosed in a family. Unlike differentiated thyroid cancer, there is no known effective systemic therapy for medullary thyroid cancer.

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