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.
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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