Outcomes and Prognostic Factors in Patients with Malignant Peripheral Nerve Sheath Tumor

Objective: To investigate and report the clinical profiles, treatment patterns, and oncologic outcomes in MPNST patients, and to identify the prognostic factors that significantly affect survival. Materials and Methods: Patients diagnosed with and treated for histologically confirmed MPNST at our institute during the January 1997 to June 2018 study period were included. Patient medical records and surgical specimens were reviewed, and study-related data was extracted and analyzed. Results: There were 27 males and 32 females with a mean age of 44 years. Most patients presented with mass and most patients were AJCC stage III. Twenty-nine percent of patients had MPNST that was associated with NF-1. At a median follow-up time, 18 patients (30.51%) suffered from local disease recurrence. Two-year and 5-year overall survival was 72% and 46%, respectively. In univariate analysis, chemotherapy treatment and positive tumor margin were adverse prognostic factors for disease-free survival. In multivariate analysis, chemotherapy treatment (hazard ratio (HR): 3.415, 95% CI: 1.367-16.021; p=0.013) and positive tumor margin (HR: 4.680, 95% CI 1.828-10.314; p=0.014) were found to be independent prognostic factors for disease-free. Conclusion: Chemotherapy treatment and positive tumor margin were identified as independent adverse prognostic factors for disease-free and overall survival, respectively. Accordingly, early detection and appropriate treatment are essential for improved patient outcome