Rhabdomyosarcoma of the ear in childhood. A report from the intergroup rhabdomyosarcoma study‐I

Twenty‐four children with rhabdomyosarcoma of the middle (22 patients) or external ear (two patients) were entered on the Intergroup Rhabdomyosarcoma Study‐I protocol from 1972 to 1978. After surgery, treatment consisted of radiotherapy plus vincristine, dactinyomycin, and cyclophosphamide, with or without Adriamycin. Nine of 19 patients (47%) who presented with localized sarcoma are free of disease at 2.2 to 6.5 years after diagnosis (median, 3.6 years). One is alive with regional recurrence at 6.7 years; another developed a contralateral cerebellar astrocytoma 4.4 years from diagnosis and died without evidence of rhabdomyosarcoma 2 months later. The other 13 children died of recurrent rhabdomyosarcoma at 5 to 25 months after diagnosis (median duration of survival, 10 months). Outcome was influenced by the presence of diagnosis of signs indicating meningeal extension; death rates were 5 of 5 patients with intracranial tumor, 3 of 4 with petrous bone erosion, 2 of 6 with facial nerve palsy, and 3 of 9 with no initial evidence of meningeal extension.