Evolving Strategies for Targeted Cancer Therapy--Past, Present, and Future
Open Access
- 6 July 2004
- journal article
- research article
- Published by Oxford University Press (OUP) in JNCI Journal of the National Cancer Institute
- Vol. 96 (13) , 980-981
- https://doi.org/10.1093/jnci/djh214
Abstract
Medullary thyroid carcinoma occurs most often as a sporadic malignancy, although in approximately 30% of cases, it occurs as part of the multiple endocrine neoplasia type 2 (MEN2) syndromes MEN2A and MEN2B or the related disease, familial medullary thyroid carcinoma. These three syndromes, inherited as autosomal dominant traits, are clinically characterized by near complete penetrance but variable expressivity. The thyroid malignancy is present in virtually all patients with MEN2A, MEN2B, and familial medullary thyroid carcinoma, and it is the most common cause of death in affected patients. Medullary thyroid carcinoma cells secrete the hormone calcitonin, and elevated plasma levels of this polypeptide, either basally or following provocation with intravenous calcium and pentagastrin, indicate the presence of medullary thyroid carcinoma, even though the tumor may not be clinically evident. The test is especially useful for evaluating patients after removal of the thyroid gland.Keywords
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