title: Bevacizumab treatment for vestibular schwannoma in a patient with neurofibromatosis type 2: hearing improvement and tumor shrinkage.
abstract: OBJECTIVE
  Neurofibromatosis type 2 (NF2) is a dominantly inherited genetic condition that clinically manifests through the appearance of multiple meningiomas, ependymomas and bilateral vestibular schwannomas (acoustic neuromas) which lead to progressive hearing loss. Neovascularization is necessary for tumor growth and is driven by tumor-produced angiogenic factors such as vascular endothelial growth factor (VEGF). Bevacizumab is a humanized monoclonal antibody that neutralizes the activity of VEGF. Recent data have shown that VEGF is produced by schwannoma tumor cells. Bevacizumab treatment in patients with NF2 who were considered poor candidates for surgery and radiation therapy was found to result in clinically meaningful hearing improvement and tumor volume reduction in previous studies.
  
  
  METHODS
  We report the case of a 40-year-old woman with sudden right-sided hearing loss. Magnetic resonance imaging (MRI) revealed multiple meningiomas and neurinomas (C2 and L5 lesions) and a right-sided acoustic neurinoma, confirming the diagnosis of NF2. Bevacizumab was given as infusion every 2 weeks at a dose of 5.0 mg/kg body weight with MRI monitoring every 6 months.
  
  
  RESULTS
  After 6 months from the start of therapy the patient reported progressive improvement of hearing response in audiometry, word recognition and face-to-face conversation. MRI evidenced reduction of the volume of the right vestibular schwannoma and the multiple meningiomas as well as attenuation of brain stem compression.
  
  
  CONCLUSIONS
  At the time of writing the patient is continuing treatment with bevacizumab without adverse events. She has good functional status and quality of life.
authors.affiliation: Unit of Oncology, University Hospital Maggiore della Carità, Novara - Italy.
authors.forename: Andrea P
authors.initials: AP
authors.lastname: Sponghini
authors.affiliation: Unit of Oncology, University Hospital Maggiore della Carità, Novara - Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Platini
authors.affiliation: Unit of Oncology, University Hospital Maggiore della Carità, Novara - Italy.
authors.forename: David
authors.initials: D
authors.lastname: Rondonotti
authors.affiliation: Department of Neurooncology, University of Turin and City of Health and Science Hospital, Turin - Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Soffietti
mesh_terms: D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D001299:Audiometry; D000068258:Bevacizumab / Q000627:therapeutic use*; D004428:Ear Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D006309:Hearing; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008577:Meningeal Neoplasms / Q000188:drug therapy; D008579:Meningioma / Q000188:drug therapy; D009442:Neurilemmoma / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016518:Neurofibromatosis 2 / Q000503:physiopathology*; D011788:Quality of Life; D016896:Treatment Outcome; D014722:Vestibule, Labyrinth / Q000473:pathology
chemical_list: D020533:Angiogenesis Inhibitors; D000068258:Bevacizumab