Purpose of review The aim of this study was to analyse the rational of the possible therapeutic approaches to thyroglossal duct cyst carcinomas (TGDCCa), especially in consideration of their potential airway involvement, discussing the most debated issues concerning employment of thyroidectomy, neck dissection and adjuvant treatments. Recent findings The literature is unanimous in defining the Sistrunk procedure as the baseline of surgical treatment of TGDCCa, and in equating the vast majority of thyroid-like TGDCCas to classic thyroid cancers from a biological point of view, while the rarer squamous cell carcinomas seem to behave more aggressively. Thyroidectomy, neck dissection and radioactive iodine treatment are considered for high-risk lesions, with the addition of customized partial resection of laryngeal cartilages when airway involvement is encountered. Furthermore, the analysis of thyroid mutational markers has promise for accurate prevision of more aggressive clinical behaviours. Summary Even if rare, clinicians should be aware of TGDCCa due to the possibility of incidental diagnosis and, in the case of more advanced clinical scenarios, for its potential airway involvement. Sistrunk procedure combined with thyroidectomy, neck dissection and adjuvant therapy provide excellent results in high-risk patients. Additional study of pathological thyroid markers in TGDCCa is desirable to allow more individualized treatments. Correspondence to Cesare Piazza, Department of Otorhinolaryngology, Head and Neck Surgery, ASST Spedali Civili of Brescia, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Piazza Spedali Civili 1, 25123 Brescia, Italy. Tel: +39 30 3995319; e-mail: cesare.piazza@unibs.it Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
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