Nowadays, with a good molecular understanding of these tumors (which includes upregulation of mitogen-activated protein kinase (RAS/MAPK), phosphoinositide 3-kinase (Pi3K), and the mammalian target of rapamycin (MTOR) pathways [99]), new evolving targeted therapy are now under investigations, and new insights into therapeutic options for MPNST will likely result [100]. Here, MTOR is linked to malignant peripheral nerve sheath tumor.