Objectives: (1) To review the epidemiology, pathophysiology, and treatment of giant cell tumor of the larynx (2) To report the first case of malignant transformation of giant cell tumor of the larynx Methods: A case of giant cell tumor of the larynx from a tertiary care university teaching hospital is presented, including the patient's clinical course and subsequent treatment. The case is discussed within the context of a review of the current literature regarding this disease entity. To our knowledge, this report represents the first case of malignant transformation of giant cell tumor of the larynx. Results: The patient underwent hemilaryngopharyngectomy with radial forearm free flap reconstruction and eleven of fifteen cycles of chemotherapy. He remains disease free at approximately 36 months follow-up. The patient is decannulated and continues to have a good voice with excellent quality of life to this day. Conclusion: Our experience demonstrates that giant cell tumor of the larynx may present as a malignant neoplasm without adversely affecting the patient's prognosis when treated aggressively with surgical resection and adjunct chemotherapy.
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