The purpose of this paper is to review the presentation and management of thyroglossal duct carcinoma. Recent articles have analyzed the value of preoperative investigation and have addressed some of the controversies in the management of such tumors; in particular, the optimal surgical management of the thyroid gland, as well as optimal management of lymph node metastases, the role of thyroid suppression therapy, and radioactive iodine therapy. Thyroglossal duct carcinoma is uncommon, occurring in approximately 1% of all thyroglossal duct cysts. It is often diagnosed incidentally after surgical excision. Ninety-four percent of carcinomas are of thyroid origin, with most being papillary in nature, and 6% are of squamous cell origin. Incidentally discovered, well-differentiated thyroid carcinoma of the thyroglossal duct, in the presence of a clinically and radiologically normal thyroid gland, can be managed adequately by the Sistrunk operation. Those patients with more advanced disease require more aggressive treatment. This may include a total thyroidectomy with or without neck dissection in addition to the Sistrunk operation, followed by radioactive iodine therapy and thyroid-stimulating hormone suppression. The prognosis is generally excellent with adequately treated disease.
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