Effect of bevacizumab on non-target intracranial meningiomas and non-vestibular schwannomas in NF2-related schwannomatosis: NF104 Journal Article


Authors: Nakhate, V.; Ly, I.; Muzikansky, A.; Rapalino, O.; Allen, J. C.; Blakeley, J. O.; Campian, J. L.; Clapp, D. W.; Dhall, G.; Jain, R. K.; Karajannis, M. A.; Packer, R. J.; Tonsgard, J.; Ullrich, N. J.; Korf, B. R.; Fisher, M. J.; Plotkin, S. R.; on behalf of the Neurofibromatosis Clinical Trials Consortium
Article Title: Effect of bevacizumab on non-target intracranial meningiomas and non-vestibular schwannomas in NF2-related schwannomatosis: NF104
Abstract: Purpose: Bevacizumab treatment is associated with imaging and hearing responses in progressive vestibular schwannoma (VS) caused by NF2-related schwannomatosis (NF2-SWN). However, its effect on co-existing intracranial non-vestibular schwannomas (NVS) and meningiomas is unclear. Methods: We retrospectively analyzed tumor volumes of non-target intracranial NVS and meningiomas in patients with NF2-SWN and progressive VS who were prospectively treated with bevacizumab for two years on the Neurofibromatosis Clinical Trials Consortium (NFCTC) trial NF104 (NCT01767792). Radiographic response (RR) or progression (PD) were defined as ≥ 20% decrease or ≥ 20% increase in tumor volume compared to baseline, respectively. All other responses were defined as stable disease. Results: A total of 40 meningiomas in eight patients and 12 NVS in six patients were evaluated across 22 enrolled trial participants. On best response analysis, RR occurred in 13% (5/40) of meningiomas and in 42% (5/12) of NVS. On a per-patient basis, RR for meningioma occurred in 38% (3/8) of patients and for NVS in 67% (4/6) of patients. RR in two NVS were durable throughout the study period. During two years of treatment, PD occurred in 55% (22/40) of meningiomas and in 8% (1/12) of NVS. Median time to tumor progression was 15 months for meningiomas and was not reached for NVS. Conclusions: We observed greater activity of bevacizumab against intracranial NVS compared to meningioma, evidenced by more favorable RR rates, durability of response, and rates of PD. Potential biological differences between meningiomas and schwannomas that underlie this differential response to bevacizumab warrant further investigation. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2025.
Keywords: adult; treatment outcome; aged; middle aged; retrospective studies; young adult; genetics; angiogenesis inhibitor; bevacizumab; follow up; follow-up studies; neurofibromatosis; skin neoplasms; pathology; diagnostic imaging; retrospective study; skin tumor; meningeal neoplasms; meningioma; drug therapy; angiogenesis inhibitors; merlin; neurofibromatoses; neurofibromin 2; neurilemoma; nf2; schwannomatosis; complication; neurilemmoma; neurofibromatosis 2; humans; human; male; female; neurofibromatosis type 2; immunological antineoplastic agent; antineoplastic agents, immunological; meningeal tumor; neurofibromatosis type 3; non-vestibular schwannoma
Journal Title: Journal of Neuro-Oncology
Volume: 173
Issue: 3
ISSN: 0167-594X
Publisher: Springer  
Date Published: 2025-07-01
Start Page: 751
End Page: 757
Language: English
DOI: 10.1007/s11060-025-05020-1
PUBMED: 40434540
PROVIDER: scopus
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PubMed record and PDF -- Source: Scopus
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