Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation

Abstract

Background: Current controversies in pediatric intracranial ependymoma include histologic categorization and management. Most of our knowledge of this disease comes from single-institution reports.

Methods: A literature search was done, covering the period 1976-1996. The aim of this review is to analyze the prognostic factors reported in the literature over the last 20 years.

Results: Forty-five series were reviewed, including more than 1,400 children. The largest series reported on 92 patients, and the accrual rate ranged from 0.32-12 patients per year. None of the prognostic factors reported achieved a consensus throughout the different series. Histology remains a major issue, and the range in the incidence of anaplastic ependymo mas (7-89%) highlights the difficulty in agreeing on a histological grading system. The role of surgery on the outcome seems to be determinant. Recent series based on homogeneous imaging-documented extents of resection strongly support the benefit of postoperative radiotherapy. The lack of a proven, effective chemotherapy regimen precludes its use except in prospective pilot studies.

Conclusions: Limited information is available from single-institution reports in ependymoma. Only large national or international studies can provide enough information to allow a multivariate analysis of the prognostic factors, and thus lead to new therapeutic proposals.