A systematic review and meta-analysis of outcomes following active surveillance, surgery and radiotherapy of meningiomas in NF2-related schwannomatosis

Abstract Background Meningiomas affect up to 80% of patients with NF2-related schwannomatosis during their lifetime. They are managed by active monitoring, surgery, and stereotactic radiosurgery (SRS). This paper aims to synthesize the existing data, evaluate outcomes, and inform future trial design. Methods Systematic review and meta-analysis conducted using the PRISMA framework. Six databases were searched from inception to September 2025. Patient demographics, intervention data, and outcomes were collected and pooled analyses performed. Studies were appraised using the NIH quality assessment tool. Results Fifteen studies with 937 patients and 3637 meningiomas were included. The pooled proportion of female patients was 59.6% (95% confidence interval [CI]: 55.4-63.7). A total of 2082 tumors were monitored (mean follow-up 5.55-9.18 years) with a weighted mean growth rate of 0.508 cm3/year (95% CI: 0.0244-0.992) (available in 748 meningiomas). The weighted pooled proportion of monitored patients who developed de novo meningiomas across 3 studies was 24.6% (95% CI: 2.73-58.7). Surgical resection was performed in 203 meningiomas, with an under-reported postintervention follow-up and a pooled risk of tumor recurrence of 12.5% (95% CI: 7.98-17.9). Stereotactic radiosurgery was used in 665 meningiomas. The pooled risk of treated tumor progression was 6.29% (95% CI: 4.57-8.25), median follow-up 3.58 to 9.25 years. The pooled local control rates at 3 and 5 years were 97.1% (95% CI: 94.7-98.8) and 91.2% (95% CI: 88.4-93.6), respectively. Conclusion NF2-associated meningiomas are challenging to manage due to their multiplicity, high growth rate, and World Health Organization grade. Active monitoring, surgery, and SRS are viable treatment options. Here, we evaluate existing outcome data to guide future trial design.