Ewing Sarcoma in the Pediatric Population: Predictors of Survival Within the United States.

Matthew E Wells, Michael D Eckhoff, William Davis, Vishwajeet Singh, Rajiv Rajani, Elizabeth M Polfer
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Abstract

Introduction: Bone and joint tumors are the third most common cause of pediatric cancer-related deaths in the United States. Although there have been improvements in survival rates among pediatric cancer patients over the past few decades, bone and joint cancers remain the exception. Considering current clinical trials involving novel targeted therapies, the establishment of updated mortality rates and predictors of survival for this cancer would be prudent. This investigation sought to determine updated 5-year survival rates and predictors of survival among pediatric Ewing sarcoma (ES) of bone treated within the United States.

Methods: The National Cancer Database was retrospectively inquired for all pediatric ES cases within the most updated bone and joint public use file available in September 2022. The reported data were truncated to only include patients with reported 5-year vital (ie, survival) status. Cox proportional hazard regression was conducted on both the truncated data and the entire cohort to validate the findings. The patients were then separated into alive versus deceased cohorts, and univariate regression analysis was done followed by multivariable regression of notable variables of interest.

Results: Overall, an aggregated 5-year survival rate of 74.5% was found in the included patient cohort. Patients with localized cancer had a comparatively improved 5-year survival rate of 84.70% as opposed to those with macrometastatic disease on presentation with a survival rate of 50.4%. Patient demographic-, tumor-, and treatment-specific variables all demonstrated an effect on survival. The multivariable predictors of worse mortality were found to include older age, larger tumor size (>8 cm), macrometastatic disease on presentation, and positive surgical margins.

Conclusion: This analysis serves to establish updated survival rates of pediatric ES treated within the United States to set standards for comparison among future studies. Continued multi-institutional and international collaboration is needed to optimize current treatment results and develop novel targeted therapies.

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小儿尤文肉瘤:美国境内的生存预测因素。
导言:在美国,骨与关节肿瘤是导致儿童癌症相关死亡的第三大常见原因。尽管过去几十年来儿科癌症患者的存活率有所提高,但骨与关节癌症仍然是个例外。考虑到目前涉及新型靶向疗法的临床试验,确定这种癌症的最新死亡率和生存预测指标将是非常谨慎的。这项调查旨在确定在美国接受治疗的小儿骨尤文肉瘤(ES)患者的最新 5 年生存率和生存预测指标:美国国家癌症数据库对2022年9月更新的骨与关节公共使用文件中的所有小儿ES病例进行了回顾性查询。对报告的数据进行了截断,仅包括报告了5年生存期(即存活率)的患者。对截断数据和整个队列进行了 Cox 比例危险回归,以验证研究结果。然后将患者分为存活组群和死亡组群,进行单变量回归分析,然后对相关显著变量进行多变量回归分析:结果:总体而言,所纳入患者队列的 5 年生存率为 74.5%。局部癌症患者的 5 年生存率为 84.70%,相比之下,出现大转移疾病的患者的 5 年生存率仅为 50.4%。患者的人口统计学变量、肿瘤变量和治疗变量都对生存率有影响。死亡率较低的多变量预测因素包括年龄较大、肿瘤较大(>8 厘米)、发病时为大转移性疾病以及手术切缘阳性:这项分析有助于确定在美国接受治疗的小儿 ES 的最新生存率,为今后的研究设定比较标准。需要继续开展多机构和国际合作,以优化目前的治疗效果并开发新型靶向疗法。
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来源期刊
CiteScore
2.60
自引率
6.70%
发文量
282
审稿时长
8 weeks
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