The Current Management of Intracranial Ependymomas in Children

E are relatively rare neuroepithelial tumors and account for 3-7% of all central nervous system (CNS) tumors. Among pediatric CNS tumors, ependymomas are the third most common intracranial tumor (6-14%) after juvenile pilocytic astrocytomas and primitive neuroectodermal tumors. The incidence in children is reported to be six times that in adults. Fifty percent of them occur under the age of five years and approximately two thirds of them occur in the infratentorial compartment. Supratentorial ependymomas occur more frequently in adults(1, 4, 8, 14, 17, 23, 28, 34, 38, 46). Surgery is the mainstay of management, and an increasing rate of complete surgical resection has been reported in recent years. Ependymomas in the posterior fossa, particularly in children, are difficult to completely resect without significant morbidity. Postoperative radiotherapy (RT) is believed to improve the prognosis of these tumors and most institutions indeed use RT as the primary adjuvant therapy for all intracranial ependymomas. Several trials of adjuvant chemotherapy have been reported, but most results have been disappointing (3, 9, 11, 35, 36, 41, 43). Although advances in diagnostic and management modalities have improved the outcome for other CNS pediatric tumors, the prognosis for intracranial epen-dymomas, particularly posterior fossa, remains dismal. This article outlines the clinciopathological features of intracranial ependymomas in children and their optimal treatment strategies.