Background <p>Neurofibromatosis type 2 (NF2) is a rare genetic disorder characterized by the development of bilateral vestibular schwannomas (VS), which can lead to progressive hearing loss and neurological deficits. Conventional treatments such as surgery and radiosurgery are often associated with significant morbidity in this population. Bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor (VEGF), has emerged as a promising non-surgical option aimed at reducing tumor volume and preserving hearing.</p> Methods <p>A comprehensive search of PubMed, Scopus, Embase, and Web of Science was conducted up to 24 January 2025, in accordance with PRISMA guidelines. Fifteen studies involving 276 patients were included. Outcomes analyzed included hearing preservation, improvement, decline, non-measurable outcomes, tumor response, and treatment-related adverse events. Random-effects meta-analyses and meta-regressions were performed.</p> Results <p>Bevacizumab was associated with a pooled hearing improvement rate of 38% (95% CI: 26–50%) and a partial tumor-volume response rate of 41% (95% CI: 29–52%). Hearing loss occurred in 24% of patients (95% CI: 12–36%), while grade III-IV adverse events were reported in 14% (95% CI: 8–20%). High heterogeneity was observed across studies. Subgroup and meta-regression analyses identified age, gender, and prior treatments as significant moderators of treatment outcomes.</p> Conclusion <p>Bevacizumab shows clinical potential for hearing preservation and tumor control in NF2-related VS. However, notable heterogeneity and risk of adverse effects underscore the need for careful patient selection and long-term monitoring. Further high-quality studies are warranted to refine treatment protocols.</p>

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Efficacy and safety of bevacizumab for vestibular schwannomas in neurofibromatosis type-2: a systematic review and meta-analysis

  • Amir Khanmirzaei,
  • Negin Safari Dehnavi,
  • Mohammad Amin Habibi,
  • Pouria Delbari,
  • Muhammad-hussain Ahmadvand,
  • Mohamad Shahir Eftekhar,
  • Fatemeh Rasouli,
  • Mahboubeh Tajvidi,
  • Amirhossein Kamroo,
  • Mehdi Haghshenas,
  • Bardia Hajikarimloo,
  • Vafa Rahimi-Movaghar

摘要

Background

Neurofibromatosis type 2 (NF2) is a rare genetic disorder characterized by the development of bilateral vestibular schwannomas (VS), which can lead to progressive hearing loss and neurological deficits. Conventional treatments such as surgery and radiosurgery are often associated with significant morbidity in this population. Bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor (VEGF), has emerged as a promising non-surgical option aimed at reducing tumor volume and preserving hearing.

Methods

A comprehensive search of PubMed, Scopus, Embase, and Web of Science was conducted up to 24 January 2025, in accordance with PRISMA guidelines. Fifteen studies involving 276 patients were included. Outcomes analyzed included hearing preservation, improvement, decline, non-measurable outcomes, tumor response, and treatment-related adverse events. Random-effects meta-analyses and meta-regressions were performed.

Results

Bevacizumab was associated with a pooled hearing improvement rate of 38% (95% CI: 26–50%) and a partial tumor-volume response rate of 41% (95% CI: 29–52%). Hearing loss occurred in 24% of patients (95% CI: 12–36%), while grade III-IV adverse events were reported in 14% (95% CI: 8–20%). High heterogeneity was observed across studies. Subgroup and meta-regression analyses identified age, gender, and prior treatments as significant moderators of treatment outcomes.

Conclusion

Bevacizumab shows clinical potential for hearing preservation and tumor control in NF2-related VS. However, notable heterogeneity and risk of adverse effects underscore the need for careful patient selection and long-term monitoring. Further high-quality studies are warranted to refine treatment protocols.