RESUMO
CONTEXT: Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft-tissue sarcoma. AIMS: The aim of this study was to analyze various prognostic factors and treatment outcome of patients with MPNST. SETTINGS AND DESIGN: This was a retrospective study. SUBJECTS AND METHODS: Ninety-two patients, who presented with MPNST at a tertiary care cancer center from 2011 to 2018, were included in this study. The median follow-up of all living patients was 33 months. Neurofibromatosis 1 (NF1) was seen in 12 (13%) patients. Sixty (65.2%) patients received curative-intent treatment. STATISTICAL ANALYSIS USED: Kaplan-Meier method was used for survival analysis. Log-rank test was used for univariate analysis, and multivariate analysis was done by Cox proportional hazard ratio method. RESULTS: The 5-year overall survival (OS) of all patients was 47.2% and the 5-year disease-free survival (DFS) of operated patients was 41.5%. On univariate analysis, association with NF1 (P = 0.009), grade (P = 0.017), and margin status (P = 0.002) had a significant effect on DFS, whereas association with NF1 (P = 0.025), metastatic disease on presentation (P < 0.0001), palliative intent of treatment (P < 0.0001), grade (P = 0.049), and margin status (P = 0.036) had a significant effect on OS. On multivariate analysis for patients who were treated with curative-intent treatment, grade (P = 0.015), and margin status (P = 0.028) had a significant effect on DFS, whereas association with NF1 (P = 0.00026) and location of tumor (P = 0.040) had a significant effect on OS. CONCLUSIONS: The presence of distant metastasis, palliative intent of treatment, association with NF1, location of the tumor in the head and neck, high tumor grade, and positive margin status were the risk factors associated with poor survival for the patients with MPNST. Wide local excision with negative resection margin is the highly recommended treatment.
