Malignant peripheral nerve sheath tumor: Transformation in a patient with neurofibromatosis type 2

AbstractMalignant peripheral nerve sheath tumor (MPNST) is a rare soft ‐tissue sarcoma with an unfavorable prognosis and limited therapeutic options. MPNSTs can be sporadic, but are often associated with neurofibromatosis (NF) 1 and usually arise from preexisting neurofibromas. MPNSTs in patients with NF2 have been reported in only exceedingly rare cases, and the mec hanisms underlying transformation into an MPNST have not been fully elucidated. Here, we describe the clinicopathological and genomic features of a peripheral nerve sheath tumor (PNST), with a primary diagnosis of a neurofibroma, as it transforms into a high‐grade MPNST in the context of NF2.
Source: Pediatric Blood and Cancer - Category: Cancer & Oncology Authors: Tags: BRIEF REPORT Source Type: research