Rhabdomyosarcoma of the ear and temporal bone

Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated. Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5 1/2 years and one at 19 years. It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.