Multicenter phase 2 trial of maintenance bevacizumab for NF2-related schwannomatosis and progressive vestibular schwannoma

A multicenter phase 2 trial in the USA evaluated bevacizumab for maintenance therapy in children and adults with NF2-related schwannomatosis (NF2-SWN) and hearing loss due to vestibular schwannomas (VS). Following induction therapy, participants received bevacizumab 5 mg/kg every three weeks for 18 months. Freedom from hearing loss in the target ear was 95% after 48 weeks of follow-up and 70% after 98 weeks. Freedom from tumor growth in the target VS was 94% after 48 weeks and 89% after 72 weeks. NF2-related QOL remained stable for 98 weeks, whereas tinnitus-related distress decreased. Maintenance bevacizumab was well tolerated, with 15% of participants discontinuating treatment due to adverse events.