Given the evidence that the SH3PXD2A-HTRA1 fusion is a potential driver present in a subset of schwannomas, Agnihotri et al., in preclinical data, provided a rationale that fusion-positive cells are potentially sensitive to MEK inhibitors and may represent a therapeutic approach for treatment-refractory fusion-positive schwannomas [108]. This evidence concerns the gene HTRA1 and schwannoma.