Rhabdomyosarcoma of the brain.

The authors present a case of primary intracranial rhabdomyosarcoma. This is only the 10th reported case and is the only one in which the patient has survived longer than 2 years. A 9-month-old boy was found to have a large mass in the right posterior fossa. Posterior fossa craniotomy revealed an unencapsulated tumour involving almost the entire right cerebellar hemisphere and extending to the right cerebellar pontine angle. Subtotal removal was done for internal decompression. On examination of the specimen by light microscopy there were definite sarcomatous features with occasional rhabdopoietic elements and many malignant giant cells. The ultrastructural appearance confirmed the diagnosis of malignant rhabdomyosarcoma. The child was treated with combination chemotherapy and cobalt-60 teletherapy. He is alive and well 2 years after operation and has no clinical evidence of recurrent disease. His physical growth and mental development are satisfactory. The response of our patient suggests that such tumours may be controlled by subtotal removal followed by radiotherapy and chemotherapy.