Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

SIMPLE SUMMARY: Malignant peripheral nerve sheath tumor (MPNST) is a soft tissue sarcoma with limited therapeutic interventions and a poor prognosis. This review summarized the current understanding of the pathogenic mechanisms behind MPNST and the latest concepts in clinical management from diagnosis to therapeutic intervention. Additionally, the developments in molecular diagnosis and targeted therapies for MPNST are highlighted. It concluded with the challenges and prospects of MPNST management. ABSTRACT: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. Although neurofibromatosis type 1 (NF1) and radiation exposure have been identified as risk factors for MPNST, the genetic and molecular mechanisms underlying MPNST pathogenesis have only lately been roughly elucidated. Plexiform neurofibroma (PN) and atypical neurofibromatous neoplasm of unknown biological potential (ANNUBP) are novel concepts of MPNST precancerous lesions, which revealed sequential mutations in MPNST development. This review summarized the current understanding of MPNST and the latest consensus from its diagnosis to treatment, with highlights on molecular biomarkers and targeted therapies. Additionally, we discussed the current challenges and prospects for MPNST management.