NF2 loss is the most common molecular driver in meningioma (~50% of grade I; higher propo...
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| ID | BMA-NF2-MENINGIOMA |
|---|---|
| Тип | Клінічна застосовність |
| Статус | переглянуто 2026-05-04 | очікує клінічного підпису | потрібне рев’ю клінічної застосовності |
| Хвороби | DIS-MENINGIOMA |
| Джерела | SRC-EANO-MENINGIOMA-2024 SRC-NCCN-CNS-2025 |
Дані про клінічну застосовність
| Біомаркер | BIO-NF2 |
|---|---|
| Варіант | NF2 loss-of-function (biallelic somatic in sporadic meningioma ~50%; germline in NF2 syndrome); merlin loss by IHC acceptable surrogate |
| Хвороба | DIS-MENINGIOMA |
| Рівень ESCAT | IIIA |
| Рекомендовані комбінації | bevacizumab 7.5–10 mg/kg IV q3w — off-label for progressive grade II-III meningioma or NF2 syndrome-related tumors; radiologic responses in ~40–50% retrospective series, selumetinib 25 mg/m² PO BID — investigational for NF2-related schwannomas (ACTR-4 regimen); not standard for meningioma |
| Підсумок доказів | NF2 loss is the most common molecular driver in meningioma (~50% of grade I; higher proportion in grade II/III). Despite its frequency, no FDA/EMA-approved targeted therapy exists specifically for NF2-mutant meningioma as of 2026. Standard treatment is maximal safe surgical resection ± radiotherapy (SRS for small residual, fractionated RT for larger/grade II-III). Systemic therapy evidence for progressive/recurrent meningioma: Selumetinib (MEK inhibitor; FDA-approved for NF1 pediatric plexiform neurofibroma): investigated in NF2-related vestibular schwannomas (ACTR-4 trial) and NF2-related meningiomas — limited hearing preservation benefit; single-agent activity modest. Bevacizumab (anti-VEGF): off-label use in progressive grade II-III meningioma or NF2-related schwannomas — radiologic responses in 40–50% in retrospective series, PFS benefit uncertain. AKT1 E17K-mutant meningioma (~10% of grade I): AKT inhibitors (capivasertib, ipatasertib) in phase II trials — not NF2-specific but relevant for co-occurring pathway alterations. SMO-mutant (~5% of meningioma): vismodegib case reports... |
Нотатки
ESCAT IIB: NF2 loss is a key molecular characterization marker for meningioma but does not yet select systemic therapy (unlike AKT1 E17K which has phase II trial data for targeted approach). Clinical priority: (1) molecular profiling of grade II-III meningioma (NF2, TRAF7, AKT1, SMO, KLF4, CDKN2A/B, TERT) to identify targetable alterations beyond NF2; (2) refer progressive/recurrent grade II-III meningioma to clinical trial (AKT inhibitor trials, CDK4/6 inhibitor trials, trabectedin trials); (3) NF2 germline testing if clinical NF2 syndrome suspected (bilateral VS, multiple meningiomas, age <25). Watch for updates: NF2-YAP pathway and FAK inhibitors (defactinib) in NF2-deficient mesothelioma and meningioma trials.
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