A patient with a laryngeal paraganglioma is presented in which overtly malignant behavior occurred despite a four-year period of symptoms prior to diagnosis. A review of the world literature reveals 52 reported cases of laryngeal paragangliomas. The incidence of malignancy in these neoplasms is often underestimated, as at least 25% of reported cases exhibited a clinically malignant natural history. We believe that the treatment of laryngeal paragangliomas should be based on the assumption that all tumors have a potential for malignant growth. Wide-field surgical excision is the applicable treatment for tumors still localized to the larynx. The results of radical neck dissection for established nodal disease are discouraging. In those patients with palpable lymph nodes, widespread dissemination has usually taken place.
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