title: Rhabdomyosarcoma of the ear in childhood. A report from the Intergroup Rhabdomyosarcoma Study-I.
abstract: 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, dactinomycin, 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.
mesh_terms: D002648:Child; D002986:Clinical Trials as Topic; D004428:Ear Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D004431:Ear, External; D004432:Ear, Middle; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000532:radiotherapy / Q000556:secondary; D008577:Meningeal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy; D009364:Neoplasm Recurrence, Local; D011879:Radiotherapy Dosage; D011897:Random Allocation; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*