Parameningeal rhabdomyosarcoma. Report of 19 cases.

Parameningeal rhabdomyosarcomas are associated with a poor long term survival. This is due to the fact that they are aggressive tumours with early involvement of the skull base. The authors report a series of 19 cases between 1984 and 1995 with a relative proportion of children, male patients and tumours of embryonal type. Three patients were operated on and only one of them was found with tumour free margins. Systemic chemotherapy using the VAC-VAd protocol was administered in 18 patients and intrathecal chemotherapy was delivered in 5 patients with involvement of the skull base. Nine patients were irradiated with doses varying from 45 to 55 Gy. Twelve patients died from 20 days to 4 years after initial treatment; 6 were lost to follow up and only one patient is living without any evidence of disease after 11 years.