Patients with neurofibromatosis type 1 (NF1) carry approximately
a 10% lifetime risk of developing a malignant peripheral nerve
sheath tumor (MPNST). Although the molecular mechanisms underlying
NF1 to MPNST malignant transformation remain unclear,
alterations of both the RAS/RAF/MAPK and PI3K/AKT/mTOR signaling
pathways have been implicated.