Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients

Otol Neurotol. 2012 Aug;33(6):1046-52. doi: 10.1097/MAO.0b013e31825e73f5.


Objective: Early studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis type 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 center

Patients: Thirty-one 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% or greater decrease in tumor volume compared with baseline.

Results: The median age was 26 years (range, 12-73 yr). 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 mo) with a total of 47 patient-years of follow-up. A hearing response occurred in 57% (13/23) of evaluable patients and a radiographic response in 55% (17/31) of target vestibular schwannomas. The median time to response was 3 months for both end points. The only clinical or radiographic feature at baseline that correlated with change in tumor volume at 3 months was the mean apparent diffusion coefficient value, a radiologic marker of edema (p = 0.036). Ninety percent 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 more than 50% of progressive vestibular schwannomas in NF2 patients. Stable or improved hearing was retained in the majority of patients.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Angiogenesis Inhibitors / adverse effects
  • Angiogenesis Inhibitors / therapeutic use*
  • Antibodies, Monoclonal, Humanized / adverse effects
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Audiometry, Pure-Tone
  • Bevacizumab
  • Child
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Hearing Loss / etiology
  • Hearing Loss / prevention & control
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neurofibromatosis 2 / complications*
  • Neuroma, Acoustic / drug therapy*
  • Neuroma, Acoustic / etiology*
  • Retrospective Studies
  • Treatment Outcome
  • Vascular Endothelial Growth Factor A / antagonists & inhibitors
  • Young Adult


  • Angiogenesis Inhibitors
  • Antibodies, Monoclonal, Humanized
  • Vascular Endothelial Growth Factor A
  • Bevacizumab