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Abstract：
BACKGROUND: Malignant peripheral nerve sheath tumours (MPNSTs) have high local recurrence (LR) rates. Literature varies on LR risk factors and treatment. This study aimed to elucidate treatment options and risk factors for first and second LRs (LR1 and LR2) in a large multicentre cohort.
METHODS: Surgically treated primary MPNSTs between 1988 and 2019 in the MONACO multicentre cohort were included. Cox regression analysed LR1 and LR2 risk factors and overall survival (OS) after LR1. Treatment of LR1 and LR2 was evaluated.
RESULTS: Among 507 patients, 28% developed LR1. Median follow-up was 66.9 months, and for survivors 111.1 months. Independent LR1 risk factors included high-grade tumours (HR 2.63; 95% c.i. 1.15 to 5.99), microscopically positive margins (HR 2.19; 95% c.i. 1.51 to 3.16) and large tumour size (HR 2.14; 95% c.i. 1.21 to 3.78). Perioperative radiotherapy (HR 0.62; 95% c.i. 0.43 to 0.89) reduced the risk. LR1 patients had poorer OS. Synchronous metastasis worsened OS (HR 1.79; 95% c.i. 1.02 to 3.14) post-LR1, while surgically treated LR was associated with better OS (HR 0.38; 95% c.i. 0.22 to 0.64) compared to non-surgical cases. Two-year survival after surgical treatment was 71% (95% c.i. 63 to 82%) versus 28% (95% c.i. 18 to 44%) for non-surgical LR1 patients. Most LR1 (75.4%) and LR2 (73.7%) patients received curative-intent treatment, often surgery alone (64.9% versus 47.4%). Radiotherapy combined with surgery was given to 11.3% of LR1 and 7.9% of LR2 patients.
CONCLUSIONS: Large, high-grade MPNSTs with R1 resections are at higher LR1 risk, potentially reduced by radiotherapy. Surgically treated recurrences may provide improved survival in highly selected cases.
METHODS: Surgically treated primary MPNSTs between 1988 and 2019 in the MONACO multicentre cohort were included. Cox regression analysed LR1 and LR2 risk factors and overall survival (OS) after LR1. Treatment of LR1 and LR2 was evaluated.
RESULTS: Among 507 patients, 28% developed LR1. Median follow-up was 66.9 months, and for survivors 111.1 months. Independent LR1 risk factors included high-grade tumours (HR 2.63; 95% c.i. 1.15 to 5.99), microscopically positive margins (HR 2.19; 95% c.i. 1.51 to 3.16) and large tumour size (HR 2.14; 95% c.i. 1.21 to 3.78). Perioperative radiotherapy (HR 0.62; 95% c.i. 0.43 to 0.89) reduced the risk. LR1 patients had poorer OS. Synchronous metastasis worsened OS (HR 1.79; 95% c.i. 1.02 to 3.14) post-LR1, while surgically treated LR was associated with better OS (HR 0.38; 95% c.i. 0.22 to 0.64) compared to non-surgical cases. Two-year survival after surgical treatment was 71% (95% c.i. 63 to 82%) versus 28% (95% c.i. 18 to 44%) for non-surgical LR1 patients. Most LR1 (75.4%) and LR2 (73.7%) patients received curative-intent treatment, often surgery alone (64.9% versus 47.4%). Radiotherapy combined with surgery was given to 11.3% of LR1 and 7.9% of LR2 patients.
CONCLUSIONS: Large, high-grade MPNSTs with R1 resections are at higher LR1 risk, potentially reduced by radiotherapy. Surgically treated recurrences may provide improved survival in highly selected cases.
摘要：
背景：恶性外周神经鞘瘤（MPNSTs）具有较高的局部复发率（LR）。关于LR危险因素和治疗的文献各不相同。本研究旨在阐明大型多中心队列中第一和第二LR（LR1和LR2）的治疗选择和风险因素。
方法：纳入了1988年至2019年MONACO多中心队列中手术治疗的原发性MPNSTs。Cox回归分析了LR1和LR2的危险因素以及LR1后的总生存期（OS）。评价LR1和LR2的治疗。
结果：在507名患者中，28%发展了LR1。中位随访时间为66.9个月，幸存者111.1个月。独立的LR1危险因素包括高级别肿瘤(HR2.63;95%c.i.1.15至5.99)，显微镜下的阳性边缘（HR2.19;95％c.i.1.51至3.16）和大肿瘤大小（HR2.14;95％c.i.1.21至3.78）。围手术期放疗（HR0.62；95％c.i.0.43至0.89）降低了风险。LR1患者的OS较差。同步转移使LR1后的OS恶化（HR1.79；95％c.i.1.02至3.14），而手术治疗的LR与非手术相比具有更好的OS（HR0.38；95％c.i.0.22至0.64）。手术治疗后的两年生存率为71％（95％c.i.63％至82％），而非手术LR1患者为28％（95％c.i.18％至44％）。大多数LR1（75.4％）和LR2（73.7％）患者接受了治愈性治疗，经常单独手术（64.9%对47.4%）。对11.3％的LR1和7.9％的LR2患者进行了放疗联合手术治疗。
结论：大，具有R1切除的高等级MPNST具有较高的LR1风险，放疗可能会减少。手术治疗的复发可以在高度选择的病例中提供改善的存活率。
方法：纳入了1988年至2019年MONACO多中心队列中手术治疗的原发性MPNSTs。Cox回归分析了LR1和LR2的危险因素以及LR1后的总生存期（OS）。评价LR1和LR2的治疗。
结果：在507名患者中，28%发展了LR1。中位随访时间为66.9个月，幸存者111.1个月。独立的LR1危险因素包括高级别肿瘤(HR2.63;95%c.i.1.15至5.99)，显微镜下的阳性边缘（HR2.19;95％c.i.1.51至3.16）和大肿瘤大小（HR2.14;95％c.i.1.21至3.78）。围手术期放疗（HR0.62；95％c.i.0.43至0.89）降低了风险。LR1患者的OS较差。同步转移使LR1后的OS恶化（HR1.79；95％c.i.1.02至3.14），而手术治疗的LR与非手术相比具有更好的OS（HR0.38；95％c.i.0.22至0.64）。手术治疗后的两年生存率为71％（95％c.i.63％至82％），而非手术LR1患者为28％（95％c.i.18％至44％）。大多数LR1（75.4％）和LR2（73.7％）患者接受了治愈性治疗，经常单独手术（64.9%对47.4%）。对11.3％的LR1和7.9％的LR2患者进行了放疗联合手术治疗。
结论：大，具有R1切除的高等级MPNST具有较高的LR1风险，放疗可能会减少。手术治疗的复发可以在高度选择的病例中提供改善的存活率。
