van Noesel MM, et al. - In this investigation, researchers reported the outcome and prognostic factors in the European Paediatric Soft Tissue Sarcoma Group (EpSSG) risk-adapted prospective study for localized pediatric malignant peripheral nerve sheath tumors (MPNST), malignant tumors arising from the nerve sheath, possibly from Schwann cells and perineural cells. Study participants included 51 patients. Data reported that the 5-year event-free survival (EFS) and overall survival (OS) were 52.9% and 62.1%, respectively. In patients with measurable disease, the response rate to chemotherapy (partial response + complete response) was 46%. An independent poor prognostic factor for OS and EFS was the presence of neurofibromatosis type 1 (NF1). Overall, the authors concluded that the outcome was outstanding for patients with resectable MPNST. The best reported outcome was the standard ifosfamide-doxorubicin for unresectable MPNST. It appears that children with NF1 disease have a worse prognosis.