Emerging systemic treatment strategies for vestibular schwannoma.

While vestibular schwannomas (VS) have traditionally been managed with surgery and radiation, systemic targeted therapies have emerged as a noninvasive means of potentially improving tumor control and functional outcomes in this patient population. In this narrative review, we explored the current landscape of systemic therapies that have been considered for the management of sporadic VS and neurofibromatosis type 2 (NF2)-VS or NF2-related schwannomatosis. In NF2-VS patients, cell proliferation has been targeted through receptor tyrosine kinase blockade and inhibition of downstream kinase activity, using lapatinib/erlotinib and everolimus respectively, and angiogenesis has been targeted through inhibition of VEGF signaling, using bevacizumab. Administration of bevacizumab demonstrated meaningful radiographic and hearing responses, while targeting cell proliferation has generally shown inconsistent results. In sporadic VS, aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) have been studied for their potential to reduce inflammation in the tumor microenvironment, thereby indirectly inhibiting cell proliferation, angiogenesis, and invasion. However, findings on their efficacy have been conflicting. Overall, while systemic therapies provide a noninvasive means of managing vestibular schwannomas by targeting proliferation, angiogenesis, and inflammation, future studies elucidating the underlying biology of these tumors are needed to optimize and implement medical therapies.