VEGF blockade with bevacizumab improved hearing in some, but not all, patients with neurofibromatosis type 2 and was associated with a reduction in the volume of most growing vestibular schwannomas ¹⁾.

Other cases support the use of bevacizumab to treat vestibular schwannomas. Given the extremely slow growth of these tumours, Eminowicz et al. note the rapidity of volume reduction following bevacizumab therapy ²⁾.

It 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 ³⁾.

In contrast to schwannomas, activation of VEGF pathway is not the primary driver of angiogenesis in meningiomas. The results suggest that a minority of NF2-associated meningiomas shrink during bevacizumab therapy and that these responses were of short duration. These results are comparable to previous studies of bevacizumab in sporadic meningiomas ⁴⁾.

The risk of severe side effects should be carefully considered and discussed with the patients prior to treatment ⁵⁾.

Treatment still requires careful monitoring of blood pressure and proteinuria, and future studies should investigate optimal dosing schedules to minimize long-term toxicity ⁶⁾.

In some NF2 patients, dose reduction of bevacizumab seems to be an effective option for managing side effects ⁷⁾.

Thorough multidisciplinary evaluation is necessary to identify the best candidates prior to treatment. It is likely that a better functional outcome would be expected if targeted therapies were discussed early in the management of the disease ⁸⁾.

The development of targeted drug therapies in addition to bevacizumab are essential to improve prognosis and quality of life in patients with NF2 given the shortcomings of surgery and radiation treatments when dealing with the multifocality of the disease ⁹⁾.