A systematic review and meta-analysis of outcomes in pediatric, recurrent ependymoma

Purpose The purpose of this study was to determine outcomes in recurrent pediatric ependymoma. Methods We performed a systematic review of PubMed, Embase, Web of Science and the Cochrane Library for studies reporting on survival outcomes for pediatric patients with recurrent ependymoma. We then performed a meta-analysis of all eligible results. Survival outcomes were identified across location of recurrence, therapy at recurrence, and age at recurrence. Results Eleven studies met final inclusion criteria. Pooled median progression free survival (PFS) from date of first recurrence was 6.7 months (95% confidence interval [95% CI] 4.7–8.8). Pooled median overall survival (OS) from date of first recurrence was 11.2 months (95% CI 6.4–16.0). Participants with supratentorial recurrences demonstrated a shorter OS of 8.3 months (95% CI 3.2–13.3) compared to 20.1 months (95% CI 8.4–31.7) for those with infratentorial recurrence. Patients who underwent surgery at recurrence had a median OS of 24.2 months (95% CI 14.2–34.1) compared to 29.2 months (95% CI 17.4–41.1) in those who received radiation compared to 19.3 months (95% CI 10.3–28.3) in those who received chemotherapy. Patients younger than age 3 years at time of recurrence demonstrated a median OS of 31.0 months (95% CI − 25.3–87.3) compared to 17.5 months (95% CI 9.9–25.2) for those that recurred beyond 3 years of age. Conclusions Our findings illustrate that children with recurrent ependymoma suffer from poor outcomes; however, these outcomes range widely depending on patient, tumor, and treatment characteristics. New therapies and treatment strategies are needed to improve outcomes in this group.