Background: No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate and LR rate for MPNST, and to assess potential risk factors for prognosis.Methods: Electronic articles published between January 1, 1966 and February 29, 2020, were searched and critically evaluated. The authors independently reviewed the abstracts and

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... tracted data for 5-year OS rate, 5-year EFS rate, LR rate, and potential risk factors for prognosis.Results: Twenty-eight literatures were finally included for meta-analysis. The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival.Conclusion: Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases and location (trunk and head & neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies.