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

E. Bouffet, G. Perilongo, A. Canete, M. Massimino, C. C. Bailey, R. Packer, A. Pierre-Kahn

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)319-331
Number of pages13
JournalMedical and Pediatric Oncology
Issue number6
Publication statusPublished - 1998


  • Brain tumors
  • Ependymoma
  • Prognostic factors

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research


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