Department of Surgical Oncology and Gastrointestinal Surgery, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
Department of Plastic and Reconstructive Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.
BJS Open. 2024 Mar 1;8(2). doi: 10.1093/bjsopen/zrae024.
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.
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.
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.
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.
恶性外周神经鞘瘤（MPNST）具有较高的局部复发（LR）率。文献中对 LR 的危险因素和治疗方法存在差异。本研究旨在阐明大型多中心队列中初次和第二次 LR（LR1 和 LR2）的治疗选择和危险因素。
纳入 1988 年至 2019 年 MONACO 多中心队列中接受手术治疗的原发性 MPNST 患者。使用 Cox 回归分析 LR1 和 LR2 的危险因素和 LR1 后的总生存（OS）。评估 LR1 和 LR2 的治疗方法。
在 507 名患者中，28%的患者发生了 LR1。中位随访时间为 66.9 个月，幸存者的随访时间为 111.1 个月。LR1 的独立危险因素包括高级别肿瘤（HR 2.63；95%置信区间 1.15 至 5.99）、显微镜下阳性切缘（HR 2.19；95%置信区间 1.51 至 3.16）和肿瘤较大（HR 2.14；95%置信区间 1.21 至 3.78）。围手术期放疗（HR 0.62；95%置信区间 0.43 至 0.89）降低了风险。LR1 患者的 OS 较差。LR1 后同步转移（HR 1.79；95%置信区间 1.02 至 3.14）使 OS 恶化，而手术治疗的 LR1 与非手术病例相比，OS 更好（HR 0.38；95%置信区间 0.22 至 0.64）。手术治疗后 2 年的生存率为 71%（95%置信区间 63%至 82%），而非手术 LR1 患者为 28%（95%置信区间 18%至 44%）。大多数 LR1（75.4%）和 LR2（73.7%）患者接受了根治性治疗，通常仅手术（64.9%比 47.4%）。LR1 患者中有 11.3%和 LR2 患者中有 7.9%接受了放疗联合手术。
大的、高级别的 MPNST 行 R1 切除后 LR1 风险较高，放疗可能降低其风险。在高度选择的病例中，手术治疗的复发可能提供更好的生存。
