Three patients with a papillary carcinoma arising in a thyroglossal duct cyst are presented and the literature is reviewed. This rare malignancy is seen mostly in women between the ages of 20 and 50 years. The distribution of carcinoma subtypes differs from that of thyroid carcinomas and thyroglossal duct carcinoma is recognized as a primary tumour. The diagnosis is seldom made pre-operatively though especially in older patients with midline swellings in the neck the diagnosis should be considered. Sistrunk's operation is the treatment of choice. In this operation the cyst, the middle part of the hyoid bone and the thyroglossal duct are removed in continuity.
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