Jan 2026: Outcomes Following Definitive Treatment of Malignant Peripheral Nerve Sheath Tumour (MPNST) are Significantly Worse for Patients with Neurofibromatosis Type 1 (NF1)

MPNSTs are aggressive soft-tissue sarcomas with a 5-year survival rate of approximately 50%, secondary to their metastatic potential and resistance to therapy. MPNSTs can arise sporadically, as a late toxicity from therapeutic radiotherapy, or in patients with neurofibromatosis type 1 (NF1). Prior studies have demonstrated conflicting results regarding the prognosis of NF1 associated MPNST compared to non-NF1 associated MPNST (NF0). We hypothesized that NF1-associated MPNSTs metastasize at a higher rate and are therefore associated with worse clinical outcomes.