Objectives: To summarize the clinical characteristics, treatment outcomes, and prognostic factors of children with non-metastatic Ewing's sarcoma (ES).
Methods: A retrospective analysis was conducted on the clinical data of 41 children with non-metastatic ES diagnosed and treated at the Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine from January 2010 to December 2018. All patients underwent chemotherapy based on the RMS-2009 protocol of the center, and local treatment such as surgery and/or radiotherapy was performed according to risk grouping. The Kaplan-Meier method was used to calculate the overall survival (OS) and event-free survival (EFS) rates. Univariate prognostic analysis was performed using the log-rank test, and multivariate analysis was conducted with Cox regression.
Results: Of the 41 children, 21 were male and 20 were female. The median age at diagnosis was 7.7 years (range: 1.2-14.6 years). The median follow-up time for patients with event-free survival was 68.1 months (range: 8.1-151.7 months). As of the last follow-up, 33 patients were in complete remission, and the overall 5-year EFS and OS rates were (78±6)% and (82±6)%, respectively. Univariate analysis by the log-rank test showed that a tumor diameter ≥8 cm, time from diagnosis to start of local treatment ≥16 weeks, and incomplete surgical resection were associated with poor prognosis (P<0.05). Multivariate Cox regression analysis indicated that incomplete surgical resection (HR=8.381, 95%CI: 1.681-41.801, P=0.010) was an independent risk factor for poor prognosis in children with ES. Secondary tumors occurred in 2 cases.
Conclusions: A comprehensive treatment strategy incorporating chemotherapy, surgery, and radiotherapy can improve the prognosis of children with ES. Poor prognosis is associated with an initial tumor diameter ≥8 cm, while complete surgical resection and early initiation of local treatment can improve outcomes.
目的: 总结儿童非转移性尤文肉瘤（Ewing sarcoma, ES）的临床特征、治疗结果和预后影响因素。方法: 对2010年1月—2018年12月上海交通大学医学院附属上海儿童医学中心诊治的41例非转移性ES初治患儿的临床资料进行回顾性分析。患儿均采用该中心横纹肌肉瘤‑2009方案进行化疗，并根据危险度分组行手术和/或放疗等局部治疗。应用Kaplan‑Meier方法计算总体生存率（overall survival, OS）、无事件生存率（event‑free survival, EFS）。采用log‑rank法行单因素预后分析，以Cox回归进行多因素分析。结果: 41例患儿中，男21例，女20例；中位发病年龄为7.7岁（范围：1.2~14.6岁）；无事件生存患儿中位随访时间为68.1个月（范围：8.1~151.7个月）。截至末次随访，33例患儿无病生存，41例患儿总体5年EFS为（78±6）%，5年OS为（82±6）%。log‑rank检验单因素分析显示，肿块直径≥8 cm、诊断至局部治疗开始时间≥16周及手术未完整切除者预后差（均P<0.05）。Cox多因素分析显示，手术未完整切除（HR=8.381，95%CI：1.681~41.801，P=0.010）是ES患儿预后差的独立危险因素。2例发生了第二肿瘤。结论: ES患儿采用化疗、手术及放疗的综合治疗预后较好；初诊时肿块直径≥8 cm者的预后较差，而手术完整切除和早期开始局部治疗可改善预后。.
Keywords: Child; Ewing's sarcoma; Prognosis; Risk Factor.