Medullary thyroid carcinoma (MTC) is a tumor that arises from thyroid C cells that
secrete calcitonin (CT). Although MTC accounts for only approximately 5% of thyroid
carcinomas in the United States [
[1]
], it has aroused considerable scientific interest because of its distinctive biochemical,
genetic, and clinical features. Although usually sporadic, some are familial tumors
that occur in unique clinical settings with an autosomal dominant mode of inheritance
[
[2]
]. The explication of the genetic basis of MTC has revolutionized management of the
familial form of this tumor and has provided insight into its pathogenesis and clinical
behavior. This article reviews the important landmarks in the history of MTC, its
molecular genetics and clinical characteristics; hereditary and sporadic forms of
the disease, biochemical and molecular diagnosis, treatment, and follow-up. Several
recent publications summarized the major advances in this field; the Seventh International
Workshop on Multiple Endocrine Neoplasia that was held in Gubbio, Italy in 1999 provided
some consensus on the diagnosis and therapy of familial MTC [
3
,
4
,
5
], although questions remain concerning the timing of thyroidectomy in certain gene
carriers.To read this article in full you will need to make a payment
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