Purpose of review: The purpose of this paper is to review the presentation and management of thyroglossal duct carcinoma.
Recent findings: 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.
Summary: 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.