关键词: Alveolar soft part sarcoma Pediatric Survival Treatment

Mesh : Humans Female Male Child Adolescent SEER Program / statistics & numerical data Sarcoma, Alveolar Soft Part / epidemiology diagnosis pathology therapy mortality Prognosis Child, Preschool Survival Rate Kaplan-Meier Estimate Infant Nomograms Proportional Hazards Models United States / epidemiology Cohort Studies

来  源:   DOI:10.1016/j.jormas.2024.101852

Abstract:
OBJECTIVE: Alveolar Soft Part Sarcoma (ASPS) is an exceedingly rare and aggressive cancer in children. Our objective was to conduct a population-based cohort study to forecast overall survival (OS) in pediatric ASPS patients.
METHODS: We utilized the Surveillance, Epidemiology, and End Results (SEER) database to identify all pediatric ASPS patients diagnosed between 1975 and 2019. Kaplan-Meier estimations were employed to construct survival curves based on various criteria. Survival curves were compared using the log-rank test. Cox proportional-hazards regression was utilized to determine variables associated with OS. Additionally, we constructed a nomogram to predict overall survival in pediatric ASPS patients.
RESULTS: A total of 103 pediatric ASPS patients were identified. Predominantly, the tumors affected females (62.2 %), and most of them located in the extremities (53.4 %). The majority of patients underwent surgery (83.5 %). Survival rates declined with increasing tumor size, and patients with localized tumors exhibited significantly better prognoses than those with distant tumors. Surgery conferred superior survival outcomes compared to no surgery. Cox proportional hazard regression analysis identified SEER stage and surgery as important independent predictors of survival.
CONCLUSIONS: Our study highlights SEER stage and surgery as key predictors of OS in pediatric ASPS, offering crucial epidemiological insights for clinical management.
摘要:
目的:肺泡软组织肉瘤(ASPS)是一种极为罕见且侵袭性的儿童癌症。我们的目的是进行一项基于人群的队列研究,以预测小儿ASPS患者的总体生存率(OS)。
方法:我们利用了监测,流行病学,和最终结果(SEER)数据库,以确定1975年至2019年之间诊断的所有儿科ASPS患者。采用Kaplan-Meier估计基于各种标准构建存活曲线。使用对数秩检验比较存活曲线。Cox比例风险回归用于确定与OS相关的变量。此外,我们构建了一个列线图来预测小儿ASPS患者的总生存期.
结果:共发现103例小儿ASPS患者。主要是,肿瘤影响女性(62.2%),其中大部分位于四肢(53.4%)。大多数患者接受了手术(83.5%)。生存率随着肿瘤大小的增加而下降,局限性肿瘤患者的预后明显优于远处肿瘤患者。与不手术相比,手术赋予了更好的生存结果。Cox比例风险回归分析确定SEER分期和手术是生存的重要独立预测因子。
结论:我们的研究强调SEER分期和手术是小儿ASPSOS的关键预测因子,为临床管理提供重要的流行病学见解。
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