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Purpose <p>Pediatric WHO grade 3 meningiomas are rare, aggressive tumors with limited population-specific outcome data. We performed an individual patient-level systematic review to characterize clinical features, management patterns, and survival outcomes in this high-risk pediatric population.</p> Methods <p>A systematic review was conducted with PRISMA guidelines. Studies reporting patient-level clinical data for pediatric patients diagnosed with WHO grade 3 meningioma were included.</p> Results <p>Fifty-seven studies encompassing 74 patients were included. Median age at diagnosis was 9&#xa0;years (range 0.38–17), with near-equal sex distribution (54% female). Median symptom duration prior to diagnosis was 2&#xa0;months (range 0.1–96). Common presenting symptoms were headache (49%), nausea/vomiting (31%), and motor deficits (26%). Molecular alterations were reported in eight cases, commonly involving <i>NF2</i> mutations (<i>n</i> = 3) and <i>YAP1:MAML2</i> fusions (<i>n</i> = 2). Gross total resection (GTR) was achieved in 60% of cases reporting extent of resection (38/63), with convexity tumors more likely to achieve GTR (<i>χ</i><sup>2</sup> = 5.15, <i>p</i> = 0.023; <i>ϕ</i> = 0.29). Kaplan-Meier analysis demonstrated significantly improved overall and progression-free survival following GTR compared with subtotal resection (OS log-rank <i>p</i> = 0.002; PFS log-rank <i>p</i> = 0.013). Adjuvant therapy was administered in 65% of cases with data, although an independent association with improved survival after adjustment for time-dependent effects was not identified in this cohort.</p> Conclusion <p>Pediatric WHO grade 3 meningiomas demonstrate aggressive clinical behavior with poor survival relative to lower grade disease. Given the vulnerable patient population, our findings highlight the need for multicenter collaboration with standardized reporting and molecular profiling to define optimal management strategies for this rare and challenging pediatric tumor.</p>

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WHO grade 3 pediatric meningioma: a systematic review of clinical features and patient-level analysis of prognostic factors

  • 期刊论文

  • 发表日期：

    2026年7月14日

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  • Hanyu Qiu,
  • Kishore Balasubramanian,
  • Daniel O’Connor,
  • Holden Husbands,
  • Christopher S. Graffeo,
  • Karl E. Balsara,
  • Jeffrey A. Zuccato,
  • Ian F. Dunn

摘要

Purpose

Pediatric WHO grade 3 meningiomas are rare, aggressive tumors with limited population-specific outcome data. We performed an individual patient-level systematic review to characterize clinical features, management patterns, and survival outcomes in this high-risk pediatric population.

Methods

A systematic review was conducted with PRISMA guidelines. Studies reporting patient-level clinical data for pediatric patients diagnosed with WHO grade 3 meningioma were included.

Results

Fifty-seven studies encompassing 74 patients were included. Median age at diagnosis was 9 years (range 0.38–17), with near-equal sex distribution (54% female). Median symptom duration prior to diagnosis was 2 months (range 0.1–96). Common presenting symptoms were headache (49%), nausea/vomiting (31%), and motor deficits (26%). Molecular alterations were reported in eight cases, commonly involving NF2 mutations (n = 3) and YAP1:MAML2 fusions (n = 2). Gross total resection (GTR) was achieved in 60% of cases reporting extent of resection (38/63), with convexity tumors more likely to achieve GTR (χ2 = 5.15, p = 0.023; ϕ = 0.29). Kaplan-Meier analysis demonstrated significantly improved overall and progression-free survival following GTR compared with subtotal resection (OS log-rank p = 0.002; PFS log-rank p = 0.013). Adjuvant therapy was administered in 65% of cases with data, although an independent association with improved survival after adjustment for time-dependent effects was not identified in this cohort.

Conclusion

Pediatric WHO grade 3 meningiomas demonstrate aggressive clinical behavior with poor survival relative to lower grade disease. Given the vulnerable patient population, our findings highlight the need for multicenter collaboration with standardized reporting and molecular profiling to define optimal management strategies for this rare and challenging pediatric tumor.