This protocol applies to vestibular schwannoma (VS) occurring in the setting of Neurofibromatosis type 2 (NF2) — a condition defined by bilateral vestibular schwannomas, or by unilateral VS presenting alongside other intracranial and/or spinal tumours.
Patients with NF2 suffer from bilateral VS or unilateral VS and other intracranial and/or spinal tumors. This multi-tumour burden, combined with the risk of progressive hearing loss, is the defining clinical challenge shaping both surveillance and treatment decisions in this population.
For patients with progressive VS in the context of NF2, an antiangiogenic therapeutic strategy represents one evidence-supported approach at this line of management.
Key clinical endpoints include reduction of tumour volume by 20% or more on volumetric radiographic assessment, alongside improvement of hearing.
DOI: 10.1093/neuonc/noz153
Patients with NF2 suffer from bilateral VS or unilateral VS and other intracranial and/or spinal tumors (see Table 1).
Bevacizumab has been successfully used for patients with progressive VS associated with NF2.
In NF2 patients with progressive VS, a prospective, multi-institutional, uncontrolled phase II study with 14 patients using 7.5 mg/kg bevacizumab administered every 3 weeks revealed a hearing improvement in 36% of patients, and there was no patient with a hearing decline in the trial period of 12 months.
Volumetric assessments demonstrated a partial radiographic response of volume reduction of 20% or more in 43% (6/14 patients), making bevacizumab a potential treatment option for NF2 patients (evidence class II, recommendation level B).
Patients experienced an improvement of hearing and objective (>20% reduced tumor volume) radiographic responses.
View source ↗