Neurofibromatosis type 2
ICD-10 Q85.0 · ICD-11 LD2D.11

Vestibular Schwannoma in NF2: Next Protocol After Surgery, Radiotherapy, or Bevacizumab Did Not Achieve Tumor Reduction or Auditory Improvement

Neurofibromatosis type 2 is characterised by bilateral vestibular schwannomas affecting the auditory nerves, resulting in hearing loss and imbalance. Treatment becomes indicated when there is risk of brainstem compression, hearing impairment, or facial nerve dysfunction.

Initial management — surgical resection of the vestibular schwannoma, radiotherapy, or bevacizumab as first-line medical therapy — targets tumor reduction and auditory improvement. This protocol addresses the situation where those goals have not been achieved and the next treatment step is required.

The next step involves investigational targeted agents from distinct mechanistic classes currently under evaluation for NF2-related vestibular schwannoma. The complete protocol specifies the agents and the clinical context in which each applies.

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References

NF2's main clinical features include bilateral vestibular schwannomas, affecting auditory nerves, causing hearing loss and imbalance.

Not all tumors require treatment, indicated when brainstem complications, hearing impairment, and/or facial nerve dysfunction are at risk.

Besides bevacizumab, other targeted therapies are under investigation for NF2-related disorder treatment, including everolimus, lapatinib, erlotinib, brigatinib, and histone deacetylase inhibitors.

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