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Xiong, ZM, ODonovan, M., Sun, L., et al

Previous attempts of targeting signaling cascades in neurofibroma microenvironment through imatinib mesylate administration, a dual SCF/cKIT inhibitor, have shown modest response rates limited only to small tumors [53] ( Cabozantinib, an inhibitor of multiple tyrosine kinases among which c-Kit, vascular endothelial growth factor (VEGF) receptor (VEGFR)2, MET, RET, FMS-related RTK 3 (FLT3) and TAM family receptors (tyrosine kinases AXL, TYRO3 and MERTK) is now under study in a phase II trial against progressive or symptomatic, inoperable PN ( as it has shown promising results in Nf1-mutant mice [54]

F.MoslehiJ

de F., Fechine, C

Current therapies based on MEK, BRAF and CDK4/6 inhibitors tend to induce resistance by activating OXPHOS and fatty acid oxidation, thus reducing the tumour mass but increasing the risk of relapse [56]