Primary Ewing's sarcoma involving the bones of the head and neck.

Ewing's sarcoma is a rare tumor of the bone. In the Intergroup Ewing's Sarcoma Study (IESS) approximately 4% of the primary bone tumors arose in the bones of the head and neck. The mean age of patients was 10.9 years; boys slightly dominated the group. Signs and symptoms were local in distribution, with a mass or swelling most frequent. As in other sites, we categorized the dominant histologic pattern as diffuse or filigree, the latter carrying a more unfavorable prognosis. The radiographic appearance may be atypical as compared to Ewing's sarcoma at other sites. Prognosis of head and neck Ewing's is significantly better than Ewing's sarcoma overall. The gnathic bones were commonly affected, yet there was no associated mortality. No patient with primary disease in the bones of the head and neck who survived for 5 years on any of the IESS protocols has subsequently died.