Prognostic factors in childhood ependymomas

Between 1975 and 1989, 45 children with newly diagnosed intracranial ependymomas were treated at the authors' institution. Patients were managed with aggressive surgical resection, followed by postoperative CT or MRI scans to evaluate the extent of resection. Most patients received involved-field radiation therapy, however 10 were treated with craniospinal axis irradiation for disseminated disease, or malignant histology. Beginning in 1983, all patients were also treated with adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatinum. Four patients died in the immediate postoperative period for an operative mortality of 8.8%. The 5-year progression-free survival for the remaining 41 patients was 36%. Tumor location had little effect on survival, as the 5-year PFS for supratentorial tumors was 26%, compared with 40% for the posterior fossa lesions (ns). Neither histologic degree of malignancy, nor use of adjuvant chemotherapy impacted on survival. The extent of surgical resection, as determined by operative reports and postoperative imaging studies, was a major determinant of outcome, as patients with total or near-total resections experienced a 5-year PFS of 60%, compared with 21% for children with partial resection or biopsy (p less than 0.01). It is concluded that the use of adjuvant chemotherapy with CCNU, vincristine, and cisplatinum does not improve progression-free survival in childhood ependymoma, and that the extent of surgical resection is a major determinant of outcome in this disease.