Abstract
Pediatric intracranial ependymoma is a rare disease representing approximately 7% of brain tumors in children aged 15 years or younger. Due to the relative rarity of these tumors, a clear standard therapy has been difficult to establish. The mainstay of treatment is surgical resection and the majority of data demonstrate improved outcome with gross total resection. The standard of care also includes postoperative radiation therapy for most patients with grade II and III tumors. Chemotherapy has been used in many capacities in this disease; however, its optimal role is yet to be defined. Current controversies such as treatment with surgery alone in completely resected tumors, use of chemotherapy for subtotally resected tumors and use of adjuvant postradiation chemotherapy are incorporated into the design of the current Children's Oncology Group clinical trial.