Division of Pediatric Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
Northwestern Quality Improvement, Research, and Education in Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Pediatr Blood Cancer. 2024 Jul;71(7):e31026. doi: 10.1002/pbc.31026. Epub 2024 Apr 28.
Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non-RCR).
This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups. Kaplan-Meier curves were generated for OS and pulmonary relapse.
Among 148 patients with metastatic EWS at diagnosis, 61 (41.2%) achieved RCR. Five-year OS was 71.2% for patients who achieved RCR, and 50.2% for those without RCR (p = .04), and in multivariable regression among patients with isolated pulmonary metastases, RCR (hazards ratio [HR] 0.42; 95% confidence interval [CI]: 0.17-0.99) and whole lung irradiation (WLI) (HR 0.35; 95% CI: 0.16-0.77) were associated with improved survival. Pulmonary relapse occurred in 57 (37%) patients, including 18 (29%) in the RCR and 36 (41%) in the non-RCR groups (p = .14). Five-year pulmonary relapse rates did not significantly differ based on RCR (33.0%) versus non-RCR (47.0%, p = .13), or WLI (38.8%) versus no WLI (46.0%, p = .32).
Patients with EWS who had isolated pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse.
本研究旨在比较初诊时发生转移的尤文肉瘤（EWS）患者中，达到快速完全缓解（RCR）的患者与诱导化疗后仍存在肺部残留结节（非 RCR）患者的总生存期（OS）和肺部复发情况。
本回顾性队列研究纳入了 2007 年至 2020 年期间在小儿外科学肿瘤协作研究机构的 19 家机构接受治疗的 20 岁以下发生转移的 EWS 患儿。采用卡方检验比较各组间的差异。绘制 OS 和肺部复发的 Kaplan-Meier 曲线。
在初诊时发生转移的 148 例 EWS 患者中，61 例（41.2%）达到 RCR。达到 RCR 的患者 5 年 OS 为 71.2%，未达到 RCR 的患者为 50.2%（p=0.04）。在单纯肺部转移的患者中，多变量回归分析显示，RCR（风险比 [HR] 0.42；95%置信区间 [CI]：0.17-0.99）和全肺照射（WLI）（HR 0.35；95% CI：0.16-0.77）与生存改善相关。57 例（37%）患者发生肺部复发，其中 RCR 组 18 例（29%），非 RCR 组 36 例（41%）（p=0.14）。基于 RCR（33.0%对非 RCR（47.0%），p=0.13）或 WLI（38.8%对无 WLI（46.0%），两组患者的 5 年肺部复发率无显著差异（p=0.32）。
初诊时发生孤立性肺部转移的 EWS 患者，如果达到 RCR 并接受 WLI，其 OS 会得到改善，尽管肺部复发率无显著差异。
