Objectives: To discuss the presentation, diagnosis, histopathology, and characteristics of clear cell carcinoma of the larnyx, including squamous and mucoepidermoid variants. Study Design: Case report and review of the literature. Methods: We report the sixth known case of clear cell carcinoma of the larynx, of squamous orgin, in a patient who presented with massive thyroid cartilage destruction and expansion in a short amount of time. We also present a review of the published literature on these lesions. Results: A 68 year old male presented with a rapidly expanding exophytic supraglottic mass with adjacent cartilage destruction. Biopsy was consistent with a stage IV clear cell carcinoma of the larynx, derived from squamous cells. He underwent total laryngectomy with bilateral neck dissections, followed by radiation and chemotherapy. He remains disease free after 12 months. Conclusions: Clear cell carcinoma (CCC) of the larynx is a rare neoplasm, with only nine cases previously reported in the literature. Of these, four were clear cell variants of mucoepidermoid or adenocarcinoma and five were of squamous cell origin. We report a sixth case of CCC of the larynx derived from squamous cells. These tumors are considered highly aggressive, with high rates of recurrence (> 85%) and short overall survival times (mean = 12 months). There is a predilection for the supraglottis, and they tend to present in elderly men, which corresponds to the findings in our patient. Surgery is the treatment of choice, and postoperative radiation/chemotherapy should be considered for this aggressive neoplasm.
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