Papillary carcinoma in a thyroglossal duct cyst: management and algorithm for treatment of a rare entity

Thyroglossal duct cyst carcinoma is a very rare finding and its presentation is similar to that of a benign cyst, which is the most common congenital lesions of the neck. The diagnosis is usually made postoperatively after histological examination. A case of a surgically treated 47-year-old patient with a papillary carcinoma in a thyroglossal duct cyst is presented. The patient underwent the Sistrunk procedure. After stratification in the proposed high risk group, subsequent total thyroidectomy and radioiodine therapy were performed successfully. The patient remains tumor-free 18 months postoperatively. Sistrunk procedure is recommended as the most promising treatment generally including total thyroidectomy with or without lymph node dissection. Subsequent radiotherapy is recommended in selected patients, depending on the classification into high- or low-risk patients. Diagnostic and therapeutic features are discussed with the current literature and a definitive algorithm for treatment based on risk group stratification is proposed.