Background: Ependymomas represent about 10% of CNS tumors in children. The proportion of cases defined as anaplastic is about 25%. Prognosis depends on extent of resection, and postoperative radiotherapy, the overall survival rate is 30-60%. Further investigations should clarify the impact of chemotherapy, histological grading, dose, and volume of radiotherapy.
Material and method: Based on historical reports, the recent literature, present guidelines, and ongoing trials an overview is provided for the management of ependymomas in childhood.
Results: Local tumor control is the most important aim. Recurrences occur predominantly at the primary tumor region. The main instrument is surgery to effect maximal tumor resection. The addition of radiotherapy could improve survival significantly from 10% to 50%. Regarding the volume of irradiation there is confidence today that local fields are sufficient for all non-disseminated ependymomas. Local dose escalation has been introduced using hyperfractionated schedules. In recent studies this has been shown to increase local control up to 70%. Regarding chemotherapy in ependymomas trials have shown limited efficacy to date. For metastatic disease standard treatment has shown to be insufficient and high dose chemotherapy regimens to increase survival are in study. In younger children radiotherapy should be delayed using early chemotherapy. With pre-irradiation chemotherapy survival rates of 63.3% for children under age of 3 were achieved.
Conclusions: At present the cooperating clinicians are optimizing treatment procedures to improve results and to reduce toxicity. In radiotherapy reduction of target volume to the involved field for all non-disseminated ependymomas as well as the introduction of hyperfractionated schedules and conformal therapy with dose escalation are important developments.