Clinical Scorecard: Survival Outcomes and Disease-Free Survival Rates in Pediatric Meningiomas: A Comprehensive Review and Meta-Analysis of Individual Patient Data

At a Glance

Key Highlights

• Pediatric meningiomas account for 1–5% of all meningiomas and differ clinically and molecularly from adult meningiomas.
• Progression-free survival (PFS) is significantly shorter in children diagnosed at ages 0–3 compared to older pediatric age groups.
• Neurofibromatosis type 2 (NF2) mutations predominate in PMs, with WHO grade and extent of resection influencing survival outcomes.

Guideline-Based Recommendations

Diagnosis

• Histopathological confirmation of cranial meningiomas in patients ≤21 years.
• Assessment of NF status, particularly NF2 mutation presence.
• Classification by WHO tumor grade (1, 2, or 3).

Management

• Aim for gross total resection (GTR, Simpson grades I-III) when feasible to improve progression-free survival.
• Consider adjuvant radiotherapy based on WHO grade and extent of resection, though data on chemotherapy are lacking.
• Tailor treatment strategies recognizing distinct pediatric tumor biology compared to adults.

Monitoring & Follow-up

• Longitudinal follow-up with progression-free survival and overall survival assessments.
• Use Kaplan–Meier survival analyses stratified by age, WHO grade, NF status, and extent of resection.
• Close monitoring especially in younger children (0–3 years) due to shorter PFS.

Risks

• Higher risk of progression in younger children (0–3 years).
• Potential for recurrence with subtotal resection (STR).
• Limited data on chemotherapy efficacy and dural attachment treatment.

Patient & Prescribing Data

Pediatric patients aged 0–21 years with sporadic or NF-associated meningiomas

Gross total resection improves progression-free survival; adjuvant radiotherapy use varies by WHO grade; chemotherapy data insufficient

Clinical Best Practices

• Perform comprehensive genetic and histopathological evaluation including NF2 mutation status.
• Prioritize gross total resection to maximize progression-free survival.
• Implement age-stratified risk assessment, recognizing younger patients have poorer PFS.
• Use multidisciplinary approaches integrating neurosurgery, radiotherapy, and genetic counseling.
• Maintain rigorous longitudinal follow-up with standardized survival outcome measures.

References

• EANO Guidelines on Meningioma Management
• NIH Quality Assessment Tool
• PRISMA Statement
• International Prospective Register of Systematic Reviews (PROSPERO)