Medullary thyroid carcinoma

Medullary thyroid carcinoma is a neuroendocrine tumor derived from the C cells of the thyroid gland and accounts for approximately 5% of all thyroid carcinomas. Approximately 30% of the cases are associated with an autosomal dominant syndrome called multiple endocrine neoplasia type 2, and the identificafion of these individuals is important because affected family members may benefit from an early diagnosis. The treatment of this disease is predominantly surgical, and the impact of radiotherapy and chemotherapy is limited. The identification of the associated molecular events has lead to the development of specific molecular targeted agents that may change the way this disease is treated in the near future.