Malignant fibrous histiocytoma degeneration in a patient with facial fibrous dysplasia.

Seventy-two patients with craniofacial fibrous dysplasia were treated during a period of 18 years. Only 1 patient (1.4%) developed malignant degeneration into malignant fibrous histiocytoma. Total excision including the gross mass and adjacent facial bone was performed at that time, however the patient refused an orbital exenteration and postoperative adjuvant therapy. Tumor recurrence developed 2 years later and the patient died. To our knowledge this is the first report describing malignant fibrous histiocytoma degeneration from a fibrous dysplasia localized to the facial bones. Although complete curative treatment could not be undertaken in this case, some conclusions can be drawn regarding the clinical course of this uncommon entity.