MPNST
Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive soft tissue sarcoma that arises from the protective coverings of peripheral nerves. It can develop de novo or from malignant transformation of a benign plexiform neurofibroma, most notably in people with neurofibromatosis type 1 (NF1).
Most patients present in adolescence to middle age. NF1-associated MPNSTs occur more frequently and tend to
Clinical features include a rapidly enlarging, painful mass along a major nerve pathway, often with new or
Imaging typically begins with magnetic resonance imaging (MRI), which shows a fusiform nerve-tumor extending along a
Treatment centers on wide surgical excision with clear margins. Adjuvant radiotherapy is commonly used to reduce
Prognosis remains guarded. Reported 5-year survival ranges from the mid-30s to around 50%, with NF1-associated tumors