Medullary thyroid carcinoma is an infrequent and unusual malignancy. Since its description 15 years ago, many new features have developed. Many biologically active compounds which are directly related to the different symptoms of the disease have been recovered from the tumors. A familial incidence is noted, and there is an increased association with other tumors and their behavior. The cell of origin is the parafollicular cell, which secretes calcitonin and is responsible for the unusual behavior of medullary thyroid carcinoma. Treatment is primiarily surgical. Four cases are presented and the syndrome reviewed.
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