Background/Objectives: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive malignancies with a challenging prognosis, especially for patients with Neurofibromatosis type 1 (NF1). Their low incidence necessitates comprehensive studies to investigate the survival outcome. Methods: We conducted a systematic review and meta-analysis, including data from 16 studies and 4265 patients, to explore surgical outcomes and survival rates, focusing on time-related outcomes, including overall survival (OS), progression-free survival (PFS), and recurrence rate. Results: The analysis revealed that the OS rate was 86% [95% CI: 75–97%] at 1 year, decreasing to 60% [95% CI: 45–75%] at 3 years, and further declining to 47% [95% CI: 35–58%] by 5 years. For PFS, the 1-year rate was 61% [95% CI: 25–98%], which remained similar at 62% [95% CI: 35–89%] for 3 and 5 years. In NF1-associated MPNSTs, the 1-year OS was relatively high at 93% [95% CI: 83–100%], but it dropped to 68% [95% CI: 53–84%] at 3 years and further to 50% [95% CI: 31–68%] at 5 years. Additionally, the hazard ratio indicated a 38% lower survival rate in NF1 patients than those with sporadic MPNSTs when data were presented in the same study. Recurrence rates were high, with 56% of patients experiencing a relapse, primarily as local recurrences (70.6%). Mortality was significant, with over 50% of patients dying within an average follow-up period of 33.45 months. Conclusions: MPNSTs, particularly in NF1 patients, are associated with poor prognosis and high recurrence rates. These results underline the necessity of targeted therapeutic strategies and improved programs for screening, mainly through a multidisciplinary approach to optimize management.
背景/目的：恶性外周神经鞘瘤（MPNSTs）是一种侵袭性强、预后不良的恶性肿瘤，尤其对于1型神经纤维瘤病（NF1）患者而言。由于其发病率较低，需要进行全面研究以探讨其生存结局。方法：我们进行了一项系统综述和荟萃分析，纳入了16项研究、共4265例患者的数据，以探讨手术结局和生存率，重点关注时间相关结局，包括总生存期（OS）、无进展生存期（PFS）和复发率。结果：分析显示，1年OS率为86%[95% CI: 75–97%]，3年下降至60%[95% CI: 45–75%]，5年进一步降至47%[95% CI: 35–58%]。PFS方面，1年率为61%[95% CI: 25–98%]，3年和5年率相似，均为62%[95% CI: 35–89%]。在NF1相关MPNSTs中，1年OS相对较高，为93%[95% CI: 83–100%]，但3年下降至68%[95% CI: 53–84%]，5年进一步降至50%[95% CI: 31–68%]。此外，当同一研究中呈现数据时，风险比表明NF1患者的生存率比散发性MPNSTs患者低38%。复发率较高，56%的患者出现复发，主要为局部复发（70.6%）。死亡率显著，超过50%的患者在平均33.45个月的随访期内死亡。结论：MPNSTs，尤其是NF1患者的MPNSTs，预后不良且复发率高。这些结果强调了制定靶向治疗策略和改进筛查方案的必要性，主要通过多学科方法优化管理。
肿瘤(癌症)患者之家