Five cases report and literature review of malignant peripheral nerve sheath tumor.

RATIONALE: Malignant Peripheral Nerve Sheath Tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. Its occurrence is closely related to genetic factors, and it is associated with Neurofibromatosis type I (NF1) in about 50% of cases. The overall incidence ranges from 3% to 10%, and it has been on the rise in recent years. METHODS: Retrospectively analyze the age of onset, tumor site, tumor size, metastasis, treatment plan, and prognosis of MPNST patients admitted to The First Affiliated Hospital of Henan Medical University from January 2018 to January 2022. RESULTS: In the past 5 years, 5 patients with MPNST aged 20-64 years were admitted to our center, including 2 males and 3 females. As of our writing of this manuscript: Of the 4 patients who underwent surgery, Case 1 patient died due to severe complications with overall survival of 11 months, Cases 2 and 4 patients are still alive with overall survival of 68 and 42 months, respectively, Case 5 patient with combined NF1 with overall survival of 18 months, and the patient in Case 3 patient was found to be inoperable due to the evaluation of condition at the time of discovery, but survived after systemic treatment with an overall survival of 45 months. All surviving patients continue to be followed up. CONCLUSION: We described the clinical features, treatment, and clinical outcomes of 5 cases of MPNST, including one case of MPNST with NF1 gene mutation. This provides valuable clinical experience for the treatment of this rare tumor.