Survival Outcomes and Disease-Free Survival in Pediatric Meningiomas

Overview

This meta-analysis of pediatric meningiomas (PMs) reveals that progression-free survival (PFS) varies significantly by age and WHO tumor grade, with younger children (0–3 years) experiencing shorter PFS. Overall survival (OS) did not differ significantly across age groups. Neurofibromatosis status and extent of resection also influence outcomes.

Background

Pediatric meningiomas are rare central nervous system tumors distinct from adult meningiomas in clinical, histopathological, and molecular characteristics. They represent 1–5% of all meningiomas and often involve NF2 mutations rather than mutations common in adults. Existing guidelines primarily address adult meningiomas, underscoring the need for pediatric-specific data. This study pools individual patient data from multiple studies to better understand survival outcomes and inform clinical management.

Data Highlights

Key Findings

• PFS is significantly shorter in children diagnosed at 0–3 years compared to older pediatric groups (p=0.04 and p=0.03).
• OS does not show significant differences across age groups (p=0.29).
• Most PMs are sporadic (87.3%) with NF2 cases evenly distributed across WHO grades.
• WHO grade distribution: Grade 1 (57.5%), Grade 2 (31.0%), Grade 3 (11.5%).
• Extent of resection (gross total vs subtotal) and adjuvant radiotherapy were analyzed but detailed data on chemotherapy were unavailable.
• PMs have a near-equal male-to-female ratio (52.1% males, 47.9% females), differing from adult meningiomas.

Clinical Implications

Clinicians should recognize that younger pediatric patients with meningiomas have a higher risk of earlier progression, necessitating closer surveillance and potentially more aggressive management. The predominance of sporadic tumors and NF2 mutation status across grades highlights the importance of genetic evaluation. Tailored treatment strategies considering WHO grade and extent of resection are essential for optimizing outcomes in this population.

Conclusion

This comprehensive meta-analysis underscores distinct survival patterns in pediatric meningiomas compared to adults, emphasizing the need for pediatric-specific clinical guidelines. Age at diagnosis and tumor grade are key prognostic factors influencing progression-free survival.

References

1. Jagtiani et al. 2024 -- Survival Outcomes and Disease-Free Survival Rates in Pediatric Meningiomas
2. Kotecha et al. 2011 -- Meta-analysis of Pediatric Meningiomas