Nakhate Vihang, Ly Ina, Muzikansky Alona, Rapalino Otto, Allen Jeffrey C, Blakeley Jaishri O, Campian Jian L, Clapp D Wade, Dhall Girish, Jain Rakesh K, Karajannis Matthias A, Packer Roger J, Tonsgard James, Ullrich Nicole J, Korf Bruce R, Fisher Michael J, Plotkin Scott R
Cancer Center and Department of Neurology, Massachusetts General Hospital, Boston, MA, USA.
Department of Radiology, Massachusetts General Hospital, Boston, MA, USA.
J Neurooncol. 2025 Jul;173(3):751-757. doi: 10.1007/s11060-025-05020-1. Epub 2025 May 28.
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.
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.
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.
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.
贝伐单抗治疗与由NF2相关神经鞘瘤病（NF2-SWN）引起的进行性前庭神经鞘瘤（VS）的影像学和听力反应相关。然而，其对并存的颅内非前庭神经鞘瘤（NVS）和脑膜瘤的影响尚不清楚。
我们回顾性分析了在神经纤维瘤病临床试验联盟（NFCTC）的NF104试验（NCT01767792）中接受贝伐单抗前瞻性治疗两年的NF2-SWN和进行性VS患者的颅内非靶标NVS和脑膜瘤的肿瘤体积。影像学反应（RR）或进展（PD）分别定义为与基线相比肿瘤体积减少≥20%或增加≥20%。所有其他反应定义为疾病稳定。
在22名入组试验参与者中，共评估了8名患者的40个脑膜瘤和6名患者的12个NVS。在最佳反应分析中，13%（5/40）的脑膜瘤和42%（5/12）的NVS出现RR。在每位患者中，38%（3/8）的患者脑膜瘤出现RR，67%（4/6）的患者NVS出现RR。两个NVS的RR在整个研究期间持续存在。在两年的治疗期间，55%（22/40）的脑膜瘤和8%（1/12）的NVS出现PD。脑膜瘤的肿瘤进展中位时间为15个月，NVS未达到。
我们观察到贝伐单抗对颅内NVS的活性高于脑膜瘤，RR率、反应持续性和PD率更有利证明了这一点。脑膜瘤和神经鞘瘤之间潜在的生物学差异是对贝伐单抗这种差异反应的基础，值得进一步研究。
