[Pediatric ependymomas: Current diagnosis and therapy]

Bull Cancer. 2016 Oct;103(10):869-879. doi: 10.1016/j.bulcan.2016.08.006. Epub 2016 Oct 4.
[Article in French]

Abstract

Ependymomas represent 10% of pediatric brain tumors. In the recent WHO 2016 classification, pathology is enriched by localization and molecular biology. Whatever the age, total removal by one or several looks when required remains a major prognostic factor. In children, focal radiation remains a standard, while the role of chemotherapy is matter of randomized studies. In infants, front line chemotherapy is the standard. Inclusion in the SIOP ependymoma II protocol is encouraged. In case of relapse, further surgery and radiation are advised, while inclusion in innovative trials including re-irradiation, and phase I-II should be encouraged. A better understanding of underlying mechanisms of ependymoma cell will provide in the close future, the key to use targeted therapies at time of relapse, and very soon as first line therapy for some subgroups of patients.

Keywords: Biologie moléculaire; Chemotherapy; Chimiothérapie; Chirurgie; Ependymoma; Molecular biology; Radiotherapy; Radiothérapie; Surgery; Épendymome.

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / genetics
  • Brain Neoplasms / pathology
  • Brain Neoplasms / therapy*
  • Child
  • Ependymoma / diagnosis*
  • Ependymoma / genetics
  • Ependymoma / pathology
  • Ependymoma / therapy*
  • Humans
  • Infant
  • Leucovorin / administration & dosage
  • Methotrexate / administration & dosage
  • Neoplasm Recurrence, Local / therapy
  • Procarbazine / administration & dosage
  • Prognosis
  • Rare Diseases / diagnosis*
  • Rare Diseases / genetics
  • Rare Diseases / pathology
  • Rare Diseases / therapy*
  • Vincristine / administration & dosage

Substances

  • Procarbazine
  • Vincristine
  • Leucovorin
  • Methotrexate

Supplementary concepts

  • SIOP-II protocol