Objective: Early studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis 2 (NF2). The aim of this study was to report extended follow-up in a larger cohort of similarly treated patients.Study Design: Retrospective study.
Setting: Tertiary referral centerPatients: 31 consecutive NF2 patients who received bevacizumab for progressive vestibular schwannomas.Main Outcome Measure: Hearing improvement, defined as an improvement in word recognition score above the 95% critical difference compared with baseline, and radiographic response, defined as a ≥20% decrease in tumor volume compared with baseline.
Results:The median age was 26 years (range, 12-73). The median volumetric tumor growth rate before treatment was 64% per year. At the time of analysis, the median duration of treatment was 14 months (range, 6-41) with a total of 45 patient-years of follow up. A hearing response occurred in 13/23 (57%) evaluable patients, and a radiographic response in 17/31 (55%) of target vestibular schwannomas. The median time to response was 3 months for both endpoints. The only clinical or radiographic feature at baseline that correlated with change in tumor volume at 3 months was the mean apparent diffusion coefficient (ADC) value, a radiologic marker of edema (p=0.036). 90% of patients had stable or improved hearing after 1 year of treatment and 61% at 3 years. 88% of patients had stable or decreased tumor size after 1 year of treatment and 54% at 3 years. Overall, treatment was well tolerated.Conclusion: Bevacizumab treatment was followed by hearing improvement and tumor shrinkage in over 50% of progressive vestibular schwannomas in NF2 patients. Stable or 2 improved hearing was retained in the majority of patients.3