Division of Hematology/Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA,
Oncology, Colorado Permanente Medical Group, Denver, Colorado, USA.
Oncology. 2024;102(1):1-8. doi: 10.1159/000533412. Epub 2023 Aug 18.
Ewing sarcoma (ES) is a small blue round cell sarcoma affecting a wide age spectrum. Clinical advances predominately stem from pediatric research consortia clinical trials. In most series, adults have poorer outcomes when compared to children. The aim of this study was to perform a detailed evaluation of factors potentially accounting for this difference.
A single institution retrospective chart review was conducted on patients with ES diagnosed from 2005 to 2015, identified using a free-text search engine with the keywords "Ewing sarcoma" as well as a corresponding pathologic database. Data were analyzed based on age, pediatric (age <18) and adult (age >18 years), using a multivariate analysis model.
Eighty-eight ES patients (34 pediatric, 54 adult) were identified with a median age of 13 (range 3-18) and 31 (range 19-70) in their respective cohorts. Five-year overall survival (OS) was higher in pediatric patients (73.5% vs. 48.1%, p = 0.0213). By stage, 5-year OS in pediatric versus adult patients was 65% versus 20% (p = 0.0530) in metastatic (n = 32) and 68.1% versus 58.8% (p = 0.278) in localized (n = 56) patients. Lung-only metastases were present in 83% of metastatic pediatric patients versus 35% of adult metastatic patients. Pediatric patients received more cycles of first-line chemotherapy (13.8 vs. 11.4, p = 0.001), independent of stage. More cycles of chemotherapy correlated with improved OS (HR: 0.864, CI: 0.773-0.967) and progression-free survival (HR: 0.897, CI: 0.808-0.996).
Outcome differences were most notable in patients with metastatic disease, although not statistically significant. Our series found differences in presentation between pediatric and adult populations with adult patients receiving fewer cycles of chemotherapy. This may suggest that both variations in underlying disease biology and potentially differences in treatment may account for outcome disparities.
尤因肉瘤（ES）是一种影响广泛年龄段的小蓝圆形细胞肉瘤。临床进展主要源自儿科研究联盟的临床试验。在大多数研究中，与儿童相比，成人的预后较差。本研究的目的是详细评估可能导致这种差异的因素。
通过使用带有关键词“Ewing sarcoma”的免费文本搜索引擎以及相应的病理数据库，对 2005 年至 2015 年间诊断为 ES 的患者进行了单中心回顾性图表审查。根据年龄（儿童[年龄＜18]和成人[年龄＞18 岁]）进行数据分析，使用多变量分析模型。
共确定 88 例 ES 患者（34 例为儿童，54 例为成人），其相应队列的中位年龄分别为 13 岁（范围 3-18 岁）和 31 岁（范围 19-70 岁）。儿童患者的 5 年总生存率（OS）更高（73.5% vs. 48.1%，p = 0.0213）。按分期，转移性（n = 32）患儿与成人患者的 5 年 OS 分别为 65%与 20%（p = 0.0530），局限性（n = 56）患儿与成人患者的 5 年 OS 分别为 68.1%与 58.8%（p = 0.278）。肺转移仅见于 83%的转移性儿科患者，而仅见于 35%的成人转移性患者。儿科患者接受的一线化疗周期数（13.8 个 vs. 11.4 个，p = 0.001）多于成人患者，而与分期无关。接受更多周期的化疗与改善 OS（HR：0.864，CI：0.773-0.967）和无进展生存期（HR：0.897，CI：0.808-0.996）相关。
在转移性疾病患者中，结果差异最为显著，尽管差异无统计学意义。本研究发现儿科和成人患者之间存在临床表现差异，成人患者接受的化疗周期数较少。这可能表明，潜在疾病生物学的变化以及潜在的治疗差异都可能导致结果差异。
