肌肉母细胞瘤（ORMS）通常表现为低风险疾病（第1期，I-III组，胚胎性RMS），并具有良好的预后。迄今为止，低风险ORMS患者的长期随访和接受最近美国儿童肿瘤学组（COG）试验治疗的ORMS亚组的结果尚未报告。鉴定了1997年至2013年在COG试验中登记的ORMS患者。收集了人口统计学信息和疾病特征。使用Kaplan-Meier方法估计以下亚组的结果：1）低风险ORMS，2）切除的低风险ORMS（I / II组），3）非低风险ORMS和4）复发ORMS。使用Kaplan-Meier方法评估无事件生存（EFS）和总生存率（OS）。作者鉴定了218名ORMS患者。大多数肿瘤为胚胎型/葡萄束样（n=169; 77.5%），<5cm（n=213；97.7%），III组（n=170；78.0%）且没有淋巴结受累（N0；n=215；98.6%）。对于192名低风险ORMS患者，10年EFS和OS率分别为85.5%（95%置信区间[CI]，77.0%-94.0%）和95.6%（95% CI，90.8%-100.0%）。那些患有I / II组低风险ORMS的人（I组的n=5; IIA组的n=39）分别具有88.0%（95% CI，72.6%-100.0%）和97.6%（95% CI，90.0%-100.0%）的10年EFS和OS率。26名非低风险ORMS患者的5年EFS和OS率分别为88.5%（95% CI，75.6%-100.0%）和95.8%（95% CI，87.7%-100.0%）。对于复发ORMS患者，从复发时间起的10年OS率为69.4%（95% CI，50.0%-88.8%）。ORMS患者在1997年至2013年的COG研究中具有有利的长期生存结果，包括那些患有低风险和非低风险疾病的患者。一部分复发ORMS患者可以实现长期生存。

Orbital rhabdomyosarcoma (ORMS) commonly presents as low-risk disease (stage 1, group I-III, embryonal RMS) with excellent outcome. Long-term follow-up of patients with low-risk ORMS and outcomes of less common subgroups of ORMS treated on recent Children's Oncology Group (COG) trials have not been reported.Patients with ORMS enrolled on COG trials from 1997 to 2013 were identified. Demographic information and disease characteristics were collected. Outcomes were determined for the following subgroups: 1) low-risk ORMS, 2) resected (group I/II) low-risk ORMS, 3) non-low-risk ORMS, and 4) recurrent ORMS. Event-free survival (EFS) and overall survival (OS) were estimated using the Kaplan-Meier method. ResultsThe authors identified 218 patients with ORMS. Most tumors were embryonal/botryoid (n = 169; 77.5%), <5 cm (n = 213; 97.7%), group III (n = 170; 78.0%), and without lymph node involvement (N0; n = 215; 98.6%). For 192 patients with low-risk ORMS, the 10-year EFS and OS rates were 85.5% (95% confidence interval [CI], 77.0%-94.0%) and 95.6% (95% CI, 90.8%-100.0%), respectively. Those with group I/II low-risk ORMS (n = 5 in group I; n = 39 in group IIA) had 10-year EFS and OS rates of 88.0% (95% CI, 72.6%-100.0%) and 97.6% (95% CI, 90.0%-100.0%), respectively. Twenty-six patients with non-low-risk ORMS had 5-year EFS and OS rates of 88.5% (95% CI, 75.6%-100.0%) and 95.8% (95% CI, 87.7%-100.0%), respectively. For patients with recurrent ORMS, the 10-year OS rate from the time of recurrence was 69.4% (95% CI, 50.0%-88.8%).Patients with ORMS had favorable long-term survival outcomes on COG studies from 1997 to 2013, including those who had both low-risk and non-low-risk disease. A significant proportion of patients with recurrent ORMS may achieve long-term survival.© 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.