Multimodality Treatment in Malignant Peripheral Nerve Sheath Tumors

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue sarcoma that originates from the peripheral nerves and is hypothesized to be of neural crest origin.1 The incidence of MPNST is 1:100.000/year and accounts for 5-10% of all soft tissue sarcomas.2 Approximately 50% of MPNSTs occur sporadically, the remaining arises secondary to prior radiation exposure and in patients with neurofibromatosis type 1 (NF1).3-5 In the NF1 patients, MPNST usually originates from plexiform neurofibromas and atypical neurofibromas. The lifetime risk of developing MPNST in these patients is around 8-13%.5-8 MPNSTs often arise from large and medium-sized nerves, located in the extremities in 33J Oncol Sci. 2020;6(1):23-8