Intraventricular Ependymoma in Pediatric Patients: A Systematic Review of Demographics, Clinical Characteristics, and Outcomes

Abstract

Background: Intraventricular neoplasms are rare occurrences observed in 5 – 7% of all primary pediatric brain tumors. Pediatric intraventricular ependymomas are a complex subset of these tumors, poorly discussed across the current literature. Although surgery is generally the accepted treatment of choice, information on clinical course and outcomes is limited to heterogeneous case reports and small case series focusing on specific histologic subtypes or ventricular locations. We conducted a systematic review on pediatric intraventricular ependymomas to survey the patient population, tumor characteristics, management strategies, and associated outcomes. Project Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched upon the PRISMA guidelines to include studies reporting pediatric patients with intraventricular ependymomas. Clinical characteristics, treatment protocols, and outcomes were analyzed. Results: A total of 9 studies with 70 patients were included. Most patients were male (54%), diagnosed at a mean age of 7 years (range, 0.2-17), and frequently exhibited nausea and vomiting (38%), headache (31%), and ataxia (25%). Tumors were predominantly located in the fourth ventricle (79%) and most tumors were WHO grade 2 (73%). Mean tumor volume was 3 cm3 (range, 0.1-13.2). Management included surgical resection (96%), radiotherapy (87%), and chemotherapy (38%). Gross total resection was achieved in 69% of cases. Cranial nerve deficit was the most common post-surgical complication (71%). Most common combination of treatment included surgical resection and radiotherapy (53%). Mean overall survival was 50 months in these patients. Conclusion/Impact: Pediatric intraventricular ependymomas are rare tumors with limited information on management strategies. The mainstay of treatment is complete surgical resection. Compared to ependymomas, intraventricular ependymomas appear to have a worse overall prognosis.