Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.

Neurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases. Due to the limited effectiveness of systemic therapy in the treatment of MPNST, the patient underwent radical surgical resection and radiotherapy. Pathological evaluation confirmed high-grade MPNST, and PET-CT imaging revealed further metastatic progression. The treatment results for our patient are compared with those of other patients with NF1 who also developed MPNST with lung metastases in the literature. The findings suggest the need for further research into personalized treatment strategies that may improve prognosis and overall survival in patients with NF1 and MPNST, with immunotherapy being a promising therapeutic option.