Non-Cochrane · intervention · PM41853812
A systematic review and meta-analysis of outcomes following active surveillance, surgery and radiotherapy of meningiomas in NF2-related schwannomatosis.
Sheppard Jack, Kannan Siddarth, Halliday Jane, et al. · Neuro-oncology advances · 2026 · PubMed · doi:10.1093/noajnl/vdag022
Findings
Population Patients with NF2-related schwannomatosis and meningiomas
14 findings
| Baseline characteristics of the cohort (no intervention) | No comparator (single-arm pooled proportion) | Proportion of female patients | Pooled proportion (%) 59.6 [55.4, 63.7] GRADE not reported 11 studies |
| Baseline characteristics of the cohort (no intervention) | No comparator (single-arm pooled proportion) | Proportion of patients with multiple meningiomas | Pooled proportion (%) 67.0 [47.0, 84.2] GRADE not reported 8 studies |
| Baseline characteristics of the cohort (no intervention) | No comparator (single-arm pooled proportion) | Proportion of meningiomas located at the skull base | Pooled proportion (%) 22.9 [19.2, 26.8] GRADE not reported 12 studies |
| Active monitoring (surveillance) | No comparator (single-arm pooled proportion) | Meningiomas progressing to require treatment Mean follow-up 5.55 to 9.18 years (3 studies reporting mean) | Pooled proportion (%) 14.1 [8.70, 20.4] Very low Downgraded: risk of bias, inconsistency 7 studies · 2082 participants |
| Active monitoring (surveillance) | No comparator (single-arm pooled proportion) | Annual meningioma growth rate | Weighted mean growth rate (cm3/year) 0.508 [0.0244, 0.992] Very low Downgraded: risk of bias, imprecision, inconsistency 4 studies · 748 participants |
| Active monitoring (surveillance) | No comparator (single-arm pooled proportion) | De novo meningioma development (patients) | Pooled proportion (%) 24.6 [2.73, 58.7] Very low Downgraded: risk of bias, imprecision, inconsistency 3 studies · 503 participants |
| Surgical resection | No comparator (single-arm pooled proportion) | Post-operative complications (patients) | Pooled proportion (%) 15.0 [0.09, 41.7] Very low Downgraded: imprecision, inconsistency 3 studies · 62 participants |
| Surgical resection | No comparator (single-arm pooled proportion) | Proportion of resected meningiomas that are high grade (WHO grade 2 and 3) | Pooled proportion (%) 26.5 [12.5, 43.5] GRADE not reported 5 studies · 159 participants |
| Surgical resection | No comparator (single-arm pooled proportion) | Local tumor recurrence after surgery | Pooled proportion (%) 12.5 [7.98, 17.9] Low Downgraded: indirectness 4 studies · 203 participants |
| Stereotactic radiosurgery (SRS) | No comparator (single-arm pooled proportion) | Progression of treated meningiomas | Pooled proportion (%) 6.29 [4.57, 8.25] Very low Downgraded: risk of bias, indirectness 5 studies · 665 participants |
| Stereotactic radiosurgery (SRS) | No comparator (single-arm pooled proportion) | Local control rate 3 years after SRS | Pooled proportion (%) 97.1 [94.7, 98.8] Moderate 3 studies · 62 participants |
| Stereotactic radiosurgery (SRS) | No comparator (single-arm pooled proportion) | Local control rate 5 years after SRS | Pooled proportion (%) 91.2 [88.4, 93.6] Low Downgraded: risk of bias 4 studies · 107 participants |
| Stereotactic radiosurgery (SRS) | No comparator (single-arm pooled proportion) | Post-treatment complications (patients) | Pooled proportion (%) 14.6 [9.39, 20.7] Very low Downgraded: risk of bias, publication bias 5 studies · 146 participants |
| Fractionated or hypofractionated radiotherapy | No comparator (single-arm pooled proportion) | Tumor control and complications | no estimate extracted No studies found 0 studies |
Included primary studies 15
Open in Studies| Study | Year | Also included by | Outcomes it informs here | Trial data |
|---|---|---|---|---|
| Aboukais 2013 Intracranial meningiomas and neurofibromatosis type 2. PMID:23558725 | 2013 | this review only | not stated | not extracted yet |
| Birckhead 2016 Gamma Knife radiosurgery for neurofibromatosis type 2-associated meningiomas: a 22-year patient series. PMID:27816997 | 2016 | this review only | not stated | not extracted yet |
| Champeaux-Depond 2020 Neurofibromatosis type 2: A nationwide population-based study focused on survival after meningioma surgery. PMID:33002675 | 2020 | this review only | not stated | not extracted yet |
| Dirks 2012 Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors. PMID:22503123 | 2012 | this review only | not stated | not extracted yet |
| Evers 2015 3D Volumetric Measurement of Neurofibromatosis Type 2-Associated Meningiomas: Association Between Tumor Location and Growth Rate. PMID:26087434 | 2015 | this review only | not stated | not extracted yet |
| Gao 2019 Efficacy and Safety of Gamma Knife Radiosurgery for Meningiomas in Patients with Neurofibromatosis Type 2: A Long-Term Follow-Up… PMID:30763746 | 2019 | this review only | not stated | not extracted yet |
| Goutagny 2012 Long-term follow-up of 287 meningiomas in neurofibromatosis type 2 patients: clinical, radiological, and molecular features. PMID:22711605 | 2012 | this review only | not stated | not extracted yet |
| Jaoude 2021 Validation of a scoring system to evaluate the risk of rapid growth of intracranial meningiomas in neurofibromatosis type 2 patients. PMID:32442973 | 2021 | this review only | not stated | not extracted yet |
| Li 2020 Clinical features of newly developed NF2 intracranial meningiomas through comparative analysis of pediatric and adult patients. PMID:32229353 | 2020 | this review only | not stated | not extracted yet |
| Liu 2015 Gamma Knife radiosurgery for meningiomas in patients with neurofibromatosis Type 2. PMID:25555193 | 2015 | this review only | not stated | not extracted yet |
| Mohammed 2022 Neurofibromatosis type 2-associated meningiomas: an international multicenter study of outcomes after Gamma Knife stereotactic radiosurgery. PMID:34144518 | 2022 | this review only | not stated | not extracted yet |
| Nowak 2015 Clinical course and management of intracranial meningiomas in neurofibromatosis type 2 patients. PMID:26652870 | 2015 | this review only | not stated | not extracted yet |
| Oyem 2022 Natural history and volumetric analysis of meningiomas in neurofibromatosis type 2. PMID:35535826 | 2022 | this review only | not stated | not extracted yet |
| Ruggieri 2005 Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: a study of 24 patients. PMID:15776319 | 2005 | this review only | not stated | not extracted yet |
| Wei 2024 Primary and salvage radiosurgery for neurofibromatosis type 2-associated meningiomas. PMID:39576970 | 2025 | this review only | not stated | not extracted yet |
Review question
| Element | Planned (methods) | Found (results) |
|---|---|---|
| Population | Patients with NF2-related schwannomatosis and meningiomas | 937 patients with 3637 meningiomas (15 studies) |
| Intervention | Active monitoring, surgery, stereotactic radiosurgery (fractionated radiotherapy also considered) | Active monitoring (2082 tumors), surgery (203 tumors), SRS (665 tumors); no fractionated radiotherapy studies included |
| Comparison | Not specified | None (no comparator groups) |
| Outcomes | Tumor growth, progression, de novo meningiomas, recurrence, complications, local control, progression-free survival, overall survival, function and quality of life | Growth rate, progression requiring treatment, de novo tumors, WHO grade, recurrence, complications, local control at 3 and 5 years, KPS (narrative) |
| Study designs | Observational studies (NIH quality tool); trials registries also searched | 15 retrospective observational studies (12 good, 3 fair on NIH tool) |
| Timing | Not specified | Monitoring mean follow-up 5.55 to 9.18 years; SRS median follow-up 3.58 to 9.25 years |
AMSTAR 2 items
Two independent extractors rated each item. Their ratings are shown side by side because they have not been reconciled yet. The overall rating follows Shea et al. 2017: one critical flaw gives Low, more than one gives Critically Low.
| Item | Extractor A | Extractor B | Agree |
|---|---|---|---|
| 1Research question includes PICO components | No | Yes | no |
| 2Protocol established before the reviewcritical | Partial Yes | Partial Yes | yes |
| 3Choice of study designs explained | No | No | yes |
| 4Comprehensive literature searchcritical | Partial Yes | Partial Yes | yes |
| 5Study selection in duplicate | Yes | Yes | yes |
| 6Data extraction in duplicate | No | No | yes |
| 7List of excluded studies with reasonscritical | No | No | yes |
| 8Included studies described in detail | Partial Yes | Yes | no |
| 9Risk of bias assessed satisfactorilycritical | RCT: N/A · NRSI: Yes | NRSI: Partial Yes · RCT: N/A | no |
| 10Funding of included studies reported | No | No | yes |
| 11Appropriate meta-analysis methodscritical | NRSI: Yes · RCT: N/A | NRSI: Yes · RCT: N/A | yes |
| 12Impact of risk of bias on the pooled results | No | No | yes |
| 13Risk of bias accounted for in interpretationcritical | Yes | Yes | yes |
| 14Heterogeneity explained | Yes | Yes | yes |
| 15Publication bias investigatedcritical | Yes | Yes | yes |
| 16Conflicts of interest reported | Yes | Yes | yes |
| Overall | Low | Low | yes |