Leiden University Medical Center, department of orthopedics, Leiden, The Netherlands.
Leiden University Mathematical Institute, Leiden, The Netherlands.
Sci Rep. 2019 Jul 29;9(1):11000. doi: 10.1038/s41598-019-46721-8.
Accurate survival estimations in Ewing sarcoma are necessary to develop risk- and response adaptive treatment strategies allowing for early decision-making. We aim to develop an easy-to-use survival estimation tool from diagnosis and surgery. A retrospective study of 1314 Ewing sarcoma patients was performed. Associations between prognostic variables at diagnosis/surgery and overall survival (OS), were investigated using Kaplan-Meier and multivariate Cox models. Predictive accuracy was evaluated by cross-validation and Harrell C-statistics. Median follow-up was 7.9 years (95%CI 7.6-8.3). Independent prognostic factors at diagnosis were age, volume, primary tumor localization and disease extent. 5 risk categories (A-E) were identified with 5-year OS of 88% (86-94), 69% (64-74), 57% (50-64), 51% (42-60) and 28% (22-34) respectively. Harrell C-statistic was 0.70. Independent prognostic factors from surgery were age, volume, disease extent and histological response. In categories A-B, 5y OS increased to 92% (87-97) and 79% (71-87) respectively for 100% necrosis and decreased to 76% (67-85) and 62% (55-69) respectively for <100% necrosis. In categories C-E, 5y OS increased to 65% (55-75), 65% (52-78) and 52% (38-66) respectively for ≥90% necrosis and decreased to 38% (22-54), 11% (0-26) and 7% (0-19) respectively for <90% necrosis. We present an easy-to-use survival estimation tool from diagnosis in Ewing sarcoma based on age, volume, primary tumor localization and disease extent. Histological response is a strong additional prognostic factor for OS.
在尤文肉瘤中进行准确的生存估计对于制定风险和反应适应性治疗策略以实现早期决策是必要的。我们旨在从诊断和手术中开发一种易于使用的生存估计工具。对 1314 例尤文肉瘤患者进行了回顾性研究。使用 Kaplan-Meier 和多变量 Cox 模型研究了诊断/手术时的预后变量与总生存（OS）之间的关系。通过交叉验证和 Harrell C 统计量评估预测准确性。中位随访时间为 7.9 年（95%CI 7.6-8.3）。诊断时的独立预后因素为年龄、体积、原发肿瘤定位和疾病程度。确定了 5 个风险类别（A-E），5 年 OS 分别为 88%（86-94）、69%（64-74）、57%（50-64）、51%（42-60）和 28%（22-34）。Harrell C 统计量为 0.70。手术时的独立预后因素为年龄、体积、疾病程度和组织学反应。在 A-B 类中，100%坏死的 5 年 OS 分别增加到 92%（87-97）和 79%（71-87），而<100%坏死的 5 年 OS 分别下降到 76%（67-85）和 62%（55-69）。在 C-E 类中，≥90%坏死的 5 年 OS 分别增加到 65%（55-75）、65%（52-78）和 52%（38-66），而<90%坏死的 5 年 OS 分别下降到 38%（22-54）、11%（0-26）和 7%（0-19）。我们提出了一种基于年龄、体积、原发肿瘤定位和疾病程度的尤文肉瘤诊断后易于使用的生存估计工具。组织学反应是 OS 的一个强有力的附加预后因素。
