Intracranial ependymomas in childhood. Survival and functional results of 47 cases.

A pediatric series of 47 intracranial ependymomas (15 supra- and 32 infratentorial) is presented. All children were operated upon between 1969 and 1979. Among these children, 35 completed the treatment with radiotherapy. The irradiation was usually limited to the intracranial content but in 7 patients, it was extended to the spinal axis as well. Malignant ependymomas represented 69% of the whole series (86% of the supratentorial and 53% of the infratentorial ependymomas). The operative mortality rate was 17%. The 5-year survival rate was 39% in the whole series and 51% when excluding postoperative mortality. Recurrences developed in 41% of cases and metastases in 20%. The study of this series and of the literature points out that: (1) metastases from supratentorial ependymomas are not frequent, almost always supratentorial and secondary to a malignant ependymoma, and (2) metastases from infratentorial ependymomas are almost always intraspinal and occur in one third of these tumors. The rate of seeding is especially high in the case of malignant infratentorial ependymomas (50%) but is also 15% in the case of benign tumors. These data lead the authors to propose a craniospinal irradiation in the case of infratentorial ependymomas whether benign or malignant, an irradiation of only the brain in the case of malignant supratentorial ependymoma, an irradiation of only the tumoral bed when ependymomas are benign and supratentorial.