Overall survival and progression-free survival in pediatric meningiomas: a systematic review and individual patient-level meta-analysis

Background: Pediatric meningiomas (PMs) are rare central nervous system tumors, accounting for 1-5% of all meningiomas, and differ from adult meningiomas in clinical, histopathological, and molecular features. Current guidelines primarily focus on adults, leaving a gap in evidence-based management for PMs. This study presents the largest meta-analysis of longitudinal individual patient data (IPD) to date, addressing progression-free survival (PFS) and overall survival (OS) in pediatric patients.

Methods: Data from 20 studies (2011-2023), including 1010 pediatric meningioma cases, were analyzed to assess PFS and OS stratified by WHO grade, NF1/NF2 status, extent of resection (EOR), and adjuvant radiotherapy. Longitudinal survival data were reconstructed from Kaplan-Meier curves using IPD extraction methods.

Results: PMs affect males and females nearly equally (52.1% vs. 47.9%). WHO grade 3 tumors had significantly shorter PFS (72.1 months) compared to grades 1 (209.8 months) and 2 (137.5 months) (p < 0.001). No significant OS difference between WHO grades 1 and 2 PMs were observed. NF1- and NF2-associated tumors showed shorter PFS (59.7 and 138.4 months) than sporadic cases (180.6 months) (p = 0.02). GTR significantly improved PFS (113.8 vs. 40.1 months, p < 0.001) and OS (602.9 vs. 173.8 months, p < 0.001). Radiotherapy enhanced PFS (72.5 vs. 23.8 months, p = 0.009) and OS (140.7 vs. 63.0 months, p = 0.002) in grade 3 tumors but not in WHO grade 2 PMs (p = 0.43).

Conclusions: This largest meta-analysis highlights the critical roles of GTR and adjuvant radiotherapy in improving outcomes for high-grade PMs and underscores the urgent need for pediatric-specific management guidelines based on robust longitudinal data.