METHODS: Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups.
RESULTS: Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, p = .018) and EFS (HR = 2.47, p = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, p = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (p < .001) and lesser baseline metastases (p = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, p = .29) or localized cohort (HR = 1.77, p = .09).
CONCLUSIONS: Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders.
方法:纳入2003年至2019年登记的ES儿童。从医疗记录中检索基线临床和治疗细节。使用多变量Cox回归确定预后因素。通过卡方和对数秩检验比较≤5岁儿童与大于5岁儿童的临床特征和结局。进行倾向评分匹配(PSM)分析以评估年龄对转移性和局部亚组生存率的影响。
结果:在859名患者中,86(10%)≤5岁(中位年龄4岁,60名男性[69.8%])。最常见的位置是四肢(37.2%),其次是胸部(27.9%)和头颈部(H&N)(22.1%);基线转移见于25例患者(29.8%).中位无事件生存期(EFS)和总生存期(OS)分别为25.6和68.7个月,分别。转移性疾病预测不良OS(风险比[HR]=2.54,p=.018)和EFS(HR=2.47,p=.007],症状持续时间≤3个月预测OS较差(HR=2.17,p=0.048)。与年龄大于5岁相比,年龄较小的儿童H&N较多,盆腔原发较少(p<.001),基线转移较少(p=.037).PSM分析未显示年龄对转移性(HR=1.59,p=0.29)或局部队列(HR=1.77,p=0.09)的OS有任何显著影响。
结论:ES≤5岁的儿童有明显的良好临床表现。然而,校正混杂因素后,年龄不是生存结局的独立预后因素.