Advancement in Genetic and Molecular Landscape of Peripheral Nerve Sheath Tumors

Peripheral nerve sheath tumors are tumors that arise throughout the central and peripheral nerve sheath tumors. Nerve sheath tumors span a spectrum of benign and malignant lesions, which include neurofibromas, schwannomas, and malignant peripheral nerve sheath tumors (MPNSTs). These tumors can arise in the setting of hereditary tumor predisposition syndromes, such as neurofibromatosis type 1 or type 2, or develop sporadically. Over the past two decades, there have been significant improvements in our understanding of the molecular alterations that drive oncogenesis. In this chapter, we will provide an overview of the current literature.