Tumeurs épendymaires intracrâniennes

Intracranial ependymoma is a rare tumour which is more frequently observed in childhood; it represents about 4% of all brain tumours. The World Health Organization (WHO) classification for ependymal tumours identifies 4 distinct entities: classic ependymoma, anaplastic ependymoma, myxopapillary ependymoma and subependymoma. Ependymoma may be an intraventricular or a paraventricular tumour, and exceptionally an intraparenchymal one. Infratentorial lesions are more frequent in children. Intracranial hypertension is the main clinical symptom. The quality of surgery has a major impact on survival. Relapses are mainly local. Focal radiation therapy is the standard adjuvant treatment, although this treatment should be avoided or differed in non anaplastic tumours with gross total resection. The role of chemotherapy is still controversial. It has been used mainly in children less than 3 years of age to delay institution of radiotherapy. The 5-year survival rate is about 60%. Identifying a subset of patients who may not need radiotherapy is the future challenge for biologists.