Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study
Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study
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围手术期化疗和放疗对四肢和躯干壁局部高级别恶性周围神经鞘瘤的作用：一项基于人群的队列研究
DOI：
10.1093/jjco/hyac178
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Tanaka S.
中科院分区：
文献类型：
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作者：
Kobayashi H;Zhang L;Okajima K;Hirai T;Tsuda Y;Ikegami M;Kawai A;Tanaka S.
背景：原发性肿瘤切除术是恶性周围神经鞘肿瘤的主要治疗方法。然而，围手术期化疗和放疗治疗恶性周围神经鞘肿瘤的疗效尚未明确。方法回顾性分析在日本登记的发生于四肢和干壁的局部恶性周围神经鞘肿瘤患者。使用Kaplan-Meier方法估计疾病特异性总生存期和局部无复发生存期。采用Cox回归模型确定预后因素。采用倾向评分匹配调整化疗组和不化疗组的特征。结果共纳入291例患者。5年疾病特异性总生存率为70.6%。疾病特异性总生存率的多因素分析显示，深部肿瘤是一个不良预后因素，但围手术期化疗与疾病特异性总生存率无相关性（风险比0.81；95%可信区间0.45-1.43，P= 0.46）。局部复发55例（19.0%），手术切缘（R1和R2）为重要危险因素。总体而言，围手术期化疗并没有延长疾病特异性总生存期（5年疾病特异性总生存期：74.1%比69.3%，P= 0.75），肿瘤大小≥5 cm组的疗效有限，但差异无统计学意义（5年疾病特异性总生存期：77.2%比68.6%，P= 0.13）。经倾向评分匹配调整后，围手术期化疗显著延长了疾病特异性总生存率（5年疾病特异性总生存率：74.9% vs. 57.1%，P= 0.03），但在局部无复发生存率中未观察到这种影响。在所有患者中，围手术期放疗与局部无复发生存无关（风险比为1.43；95%可信区间为0.78-2.62，P= 0.25）。结论围手术期化疗对局限性周围神经鞘恶性肿瘤患者疾病特异性总生存率的影响有限。
BackgroundPrimary tumor resection is the mainstay of treatment for malignant peripheral nerve sheath tumors. However, the efficacy of perioperative chemotherapy and radiotherapy for malignant peripheral nerve sheath tumors has not been elucidated.MethodsThis retrospective analysis based on a Japanese registry included patients with localized malignant peripheral nerve sheath tumors arising at the extremities and trunk wall. Disease-specific overall survival and local recurrence-free survival were estimated using the Kaplan–Meier method. A Cox regression model was used to identify prognostic factors. Characteristics of groups with or without chemotherapy were adjusted using propensity score matching.ResultsIn total, 291 patients were included. The 5-year disease-specific overall survival rate was 70.6%. Multivariate analysis of disease-specific overall survival revealed that deep-seated tumors were a poor prognostic factor, but perioperative chemotherapy was not associated with disease-specific overall survival (hazard ratio, 0.81; 95% confidence interval, 0.45–1.43,P= 0.46). Local recurrence was observed in 55 patients (19.0%), and surgical margins (R1 and R2) were significant risk factors. Overall, perioperative chemotherapy did not prolong disease-specific overall survival (5-year disease-specific overall survival: 74.1% vs. 69.3%,P= 0.75) and had limited efficacy in the group with tumor size ≥ 5 cm, although the difference was not statistically significant (5-year disease-specific overall survival: 77.2% vs. 68.6%, respectively,P= 0.13). After adjustment by propensity score matching, perioperative chemotherapy significantly prolonged disease-specific overall survival (5-year disease-specific overall survival: 74.9% vs. 57.1%,P= 0.03), but this effect was not observed in local recurrence-free survival. In all patients, perioperative radiotherapy did not correlate with local recurrence-free survival (hazard ratio, 1.43; 95% confidence interval 0.78–2.62,P= 0.25).ConclusionsPerioperative chemotherapy had limited efficacy for disease-specific overall survival in patients with localized malignant peripheral nerve sheath tumors.