The management and outcome of 24 children with intracranial ependymomas (22 benign and well differentiated) treated over a 10-year period, 1979-1988, and with a minimum 5-year follow-up were analysed at Great Ormond Street Hospital, London, UK.
The management and outcome of 24 children with intracranial ependymomas (22 benign and well differentiated) treated over a 10-year period, 1979-1988, and with a minimum 5-year follow-up were analysed at Great Ormond Street Hospital, London, UK. Of 16 with infratentorial ependymomas, 4 were alive, all 4 having total resection and 3 having craniospinal radiotherapy in addition. Of 12 who died, 5 had total resections and 4 had radiotherapy. All 7 with incomplete resections had died within 5 years despite radiotherapy and chemotherapy as well in some. Ventriculo-peritoneal shunting for hydrocephalus was required in 8 of 17 patients with posterior fossa ependymomas. In the supratentorial group of 7 patients, only one was alive and tumor-free at 5 years. No metastases were detected by myelography in 9 patients examined. [
COMMENT. In posterior fossa ependymomas an apparent total resection is associated with a better outcome but may be followed by local recurrence. Partial resection carries a very poor outcome, even with radiotherapy. Supratentorial ependymomas have a worse prognosis than the infratentorial group. In general, intracranial ependymomas in childhood have a very poor prognosis. Long-term survival may be achieved only after complete resection followed by radiotherapy, with its known effects on intellectual function and school achievement. Spinal metastases are unusual and prophylactic spinal irradiation in benign ependymoma is not recommended. Multicenter studies including chemotherapy are indicated.