Chondrosarcoma of the epiglottis is extremely rare and there are no previously reported cases of this neoplasm with regional or distant metastases. The first case of this entity and its histopathologic considerations are presented. Accurate grading of this malignancy is essential, since a more poorly differentiated pathologic condition in these tumors indicates aggressive biologic properties consequently leading to frequent local recurrences and metastasis. This is evident in the present case in which, despite being free of local or regional recurrence for 3 years following a supraglottic laryngectomy and bilateral neck dissections, the patient developed pulmonary metastases. We support the use of conservative laryngeal surgery and modified neck dissections for this disease rather than partial resections or debulking procedures, since the histopathologic condition may not be fully recognized without microscopic analysis.

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