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The Oncologist, Vol. 13, No. 5, 539-547, May 2008; doi:10.1634/theoncologist.2007-0239
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 Current Management of Medullary Thyroid Cancer
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Endocrinology

Current Management of Medullary Thyroid Cancer

Rebecca S. Sippel, Muthusamy Kunnimalaiyaan, Herbert Chen

Department of Surgery, Section of Endocrine Surgery, University of Wisconsin, Madison, Wisconsin, USA

Key Words. Thyroid neoplasm • Medullary thyroid cancer • Multiple endocrine neoplasia • RET protein

Correspondence: Rebecca Sippel, M.D., Department of Surgery, University of Wisconsin, H4/755 CSC, 600 Highland Avenue, Madison, Wisconsin 53792-7375, USA. Telephone: 608-263-1387; Fax: 608-263-7652; e-mail: sippel{at}surgery.wisc.edu

Received December 6, 2007; accepted for publication March 14, 2008.

Disclosure: No potential conflicts of interest were reported by the authors, planners, reviewers, or staff managers of this article.

Medullary thyroid cancer accounts for 5%–10% of all thyroid cancers. The majority of medullary thyroid cancers are sporadic, but 20% of cases are a result of a germline mutation in the ret proto-oncogene. Hereditary medullary thyroid cancer can be seen as part of the multiple endocrine neoplasia syndrome type 2A or 2B or as part of familial medullary thyroid cancer. This article discusses the current methods available for the diagnosis and evaluation of a patient with suspected medullary thyroid cancer. The management of medullary thyroid cancer is predominantly surgical excision, consisting of a total thyroidectomy and lymph node dissection. The extent and timing of surgical excision are discussed. Systemic therapeutic options are limited for medullary thyroid cancer, but several therapeutic targets show promise for the development of new therapies in the future.




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