Journal Article

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

Plotkin, Scott R., Rapalino, Otto, Dhall, Girish, Fisher, Michael J., Nakhate, Vihang, Jain, Rakesh K., Blakeley, Jaishri O., Karajannis, Matthias A., Korf, Bruce R., Packer, Roger J., Tonsgard, James, Ullrich, Nicole J., Ly, Ina, Clapp, D. Wade, Allen, Jeffrey C., Campian, Jian L., Muzikansky, Alona

2025

Overview

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.

• Publisher: Springer US,Springer Nature B.V
• Subject: Adult / Aged / Angiogenesis Inhibitors - therapeutic use / Antineoplastic Agents, Immunological - therapeutic use / Bevacizumab / Bevacizumab - therapeutic use / Case Study / Clinical trials / Consortia / Female / Follow-Up Studies / Genetic disorders / Hearing loss / Humans / Male / Medicine / Medicine & Public Health / Meningeal Neoplasms - complications / Meningeal Neoplasms - drug therapy / Meningeal Neoplasms - pathology / Meningioma / Meningioma - complications / Meningioma - diagnostic imaging / Meningioma - drug therapy / Meningioma - pathology / Middle Aged / Neurilemmoma - complications / Neurilemmoma - drug therapy / Neurilemmoma - pathology / Neurofibromatoses - complications / Neurofibromatoses - drug therapy / Neurofibromatoses - pathology / Neurofibromatosis / Neurofibromatosis 2 - complications / Neurofibromatosis 2 - drug therapy / Neurofibromin 2 - genetics / Neurology / Oncology / Patients / Pediatrics / Retrospective Studies / Schwann cells / Skin Neoplasms - complications / Skin Neoplasms - drug therapy / Skin Neoplasms - pathology / Software / Treatment Outcome / Tumors / Vestibular system / Young Adult