NF1 and malignant peripheral nerve sheath tumor: The main risk factors for the development of MPNST are existing benign plexiform neurofibromas (PNs),2 prior radiation treatment,3 and large germline mutations involving the entire NF1 gene (microdeletions) and surrounding genes.4 In this review, we will discuss the pathophysiology, diagnostic workup, current treatment options, and clinical trials for MPNST.