Malignant fibrous xanthoma (xanthofibrosarcoma) of the larynx

The xanthofibrosarcoma is a malignant variant of fibrous xanthoma. It is a rare tumor that derives from histiocytes. A case believed to be the first xanthofibrosarcoma reported in the larynx is presented. Differentiation between this tumor and benign lesion was difficult. The tumor proved malignant by metastasizing. Although few fibrous xanthomas become malignant they should raise a high index of suspicion when they are large, have infiltrative margins, and tend to recur locally. Treatment is by wide surgical excision. Close follow-up is mandatory. The prognosis once metastases appear is extremely poor.