PDF(Pubmed)
Abstract：
OBJECTIVE: This study sought to investigate the role of radiotherapy (RT) in addition to surgery for oncological outcomes in patients with malignant peripheral nerve sheath tumors (MPNST).
METHODS: In this single-center, retrospective cohort study, histopathologically confirmed MPNST were analyzed. Local control (LC), overall survival (OS), and distant metastasis-free survival (DMFS) were assessed using the Kaplan-Meier estimator. Multivariable Cox regression analysis was performed to identify factors associated with LC, OS, and DMFS.
RESULTS: We included 57 patients with a median follow-up of 20.0 months. Most MPNSTs were located deeply (87.5%), were larger than 5 cm (55.8%), and had high-grade histology (78.7%). Seventeen patients received surgery only, and 25 patients received surgery and pre- or postoperative RT. Median LC, OS, and DMFS after surgery only were 8.7, 25.5, and 22.0 months; after surgery with RT, the median LC was not reached, while the median OS and DMFS were 111.5 and 69.9 months. Multivariable Cox regression of LC revealed a negative influence of patients presenting with local disease recurrence compared to patients presenting with an initial primary diagnosis of localized MPNST (hazard ratio: 8.86, p = 0.003).
CONCLUSIONS: The addition of RT to wide surgical excision appears to have a beneficial effect on LC. Local disease recurrence at presentation is an adverse prognostic factor for developing subsequent local recurrences. Future clinical and translational studies are warranted to identify molecular targets and find effective perioperative combination therapies with RT to improve patient outcomes.
METHODS: In this single-center, retrospective cohort study, histopathologically confirmed MPNST were analyzed. Local control (LC), overall survival (OS), and distant metastasis-free survival (DMFS) were assessed using the Kaplan-Meier estimator. Multivariable Cox regression analysis was performed to identify factors associated with LC, OS, and DMFS.
RESULTS: We included 57 patients with a median follow-up of 20.0 months. Most MPNSTs were located deeply (87.5%), were larger than 5 cm (55.8%), and had high-grade histology (78.7%). Seventeen patients received surgery only, and 25 patients received surgery and pre- or postoperative RT. Median LC, OS, and DMFS after surgery only were 8.7, 25.5, and 22.0 months; after surgery with RT, the median LC was not reached, while the median OS and DMFS were 111.5 and 69.9 months. Multivariable Cox regression of LC revealed a negative influence of patients presenting with local disease recurrence compared to patients presenting with an initial primary diagnosis of localized MPNST (hazard ratio: 8.86, p = 0.003).
CONCLUSIONS: The addition of RT to wide surgical excision appears to have a beneficial effect on LC. Local disease recurrence at presentation is an adverse prognostic factor for developing subsequent local recurrences. Future clinical and translational studies are warranted to identify molecular targets and find effective perioperative combination therapies with RT to improve patient outcomes.
摘要：
目的：本研究旨在探讨放疗（RT）以及手术对恶性外周神经鞘瘤（MPNST）患者肿瘤预后的作用。
方法：在这个单中心，回顾性队列研究，对组织病理学证实的MPNST进行分析.本地控制（LC），总生存期（OS），和无远处转移生存期(DMFS)使用Kaplan-Meier估计量进行评估。进行多变量Cox回归分析以确定与LC相关的因素，操作系统,和DMFS。
结果：我们纳入了57例患者，中位随访时间为20.0个月。大多数MPNST位于较深（87.5%），大于5厘米(55.8%)，组织学分级高(78.7%)。17名患者只接受了手术，25例患者接受手术和术前或术后RT。中值LC,操作系统,术后DMFS分别为8.7、25.5和22.0个月；RT术后，未达到LC中位数，而中位OS和DMFS分别为111.5和69.9个月.LC的多变量Cox回归显示，与最初诊断为局部MPNST的患者相比，局部疾病复发的患者具有负面影响（风险比：8.86，p=0.003）。
结论：在广泛的手术切除中添加RT似乎对LC具有有益的作用。出现时局部疾病复发是发展后续局部复发的不良预后因素。未来的临床和转化研究有必要确定分子靶标，并找到有效的围手术期联合治疗与RT以改善患者预后。
方法：在这个单中心，回顾性队列研究，对组织病理学证实的MPNST进行分析.本地控制（LC），总生存期（OS），和无远处转移生存期(DMFS)使用Kaplan-Meier估计量进行评估。进行多变量Cox回归分析以确定与LC相关的因素，操作系统,和DMFS。
结果：我们纳入了57例患者，中位随访时间为20.0个月。大多数MPNST位于较深（87.5%），大于5厘米(55.8%)，组织学分级高(78.7%)。17名患者只接受了手术，25例患者接受手术和术前或术后RT。中值LC,操作系统,术后DMFS分别为8.7、25.5和22.0个月；RT术后，未达到LC中位数，而中位OS和DMFS分别为111.5和69.9个月.LC的多变量Cox回归显示，与最初诊断为局部MPNST的患者相比，局部疾病复发的患者具有负面影响（风险比：8.86，p=0.003）。
结论：在广泛的手术切除中添加RT似乎对LC具有有益的作用。出现时局部疾病复发是发展后续局部复发的不良预后因素。未来的临床和转化研究有必要确定分子靶标，并找到有效的围手术期联合治疗与RT以改善患者预后。
