Ahrens S, Hoffmann C, Jabar S, Braun-Munzinger G, Paulussen M, Dunst J, Rübe C, Winkelmann W, Heinecke A, Göbel U, Winkler K, Harms D, Treuner J, Jürgens H
Department of Pediatric Hematology/Oncology, University Children's Hospital, Münster, Germany.
Med Pediatr Oncol. 1999 Mar;32(3):186-95. doi: 10.1002/(sici)1096-911x(199903)32:3<186::aid-mpo5>3.0.co;2-d.
The Cooperative Ewing Sarcoma Study (CESS 86), conducted by the German Society of Pediatric Oncology and Hematology (GPOH), was planned on the basis of the results of the preceding CESS 81 study. The prognostic significance of tumor volume in localized Ewing sarcoma of bone was well documented in the CESS 81 trial. As a consequence, the treatment intensity was adapted to volume in the follow-up CESS 86 trial: the four-drug combination used in CESS 81 was amended for patients with large tumor volume (> or = 100 ml), where ifosfamide was substituted for cyclophosphamide.
From January 1986 to June 1991, 177 protocol patients with localized Ewing sarcoma of bone were registered in CESS 86. The prognostic implication of tumor volume and several covariates was evaluated using Kaplan-Meier life table analysis and Cox's proportional hazard model.
The estimated 5- and 8-year event-free survival (EFS) rates were both 59%. Age, gender, tumor site, and a tumor volume of 100 ml did not distinguish groups of patients with different prognosis. However, the prognosis of patients with tumors >200 ml (8-year EFS rate: 42%) was significantly inferior compared to patients with tumors both of 100 to 200 ml (70%) and of <100 ml (63%). In contrast to CESS 81, the histological response to chemotherapy was no longer a significant prognostic factor (EFS: 64% for good and 50% for poor responders, respectively).
Despite risk-adapted treatment intensity, tumor volume retained its prognostic significance; the cut point, however, was shifted toward larger volumes.
德国儿科肿瘤学和血液学协会（GPOH）开展的尤文肉瘤协作研究（CESS 86）是在前一项CESS 81研究结果的基础上规划的。在CESS 81试验中，肿瘤体积对局限性骨尤文肉瘤的预后意义有充分记录。因此，在后续的CESS 86试验中，治疗强度根据体积进行了调整：对于肿瘤体积较大（≥100 ml）的患者，修改了CESS 81中使用的四联药物组合，用异环磷酰胺替代了环磷酰胺。
1986年1月至1991年6月，177例符合方案的局限性骨尤文肉瘤患者被纳入CESS 86研究。使用Kaplan-Meier生存表分析和Cox比例风险模型评估肿瘤体积及几个协变量的预后意义。
估计的5年和8年无事件生存率（EFS）均为59%。年龄、性别、肿瘤部位以及100 ml的肿瘤体积并不能区分不同预后的患者组。然而，肿瘤>200 ml的患者（8年EFS率：42%）的预后明显差于肿瘤体积在100至200 ml（70%）和<100 ml（63%）的患者。与CESS 81不同，化疗的组织学反应不再是一个显著的预后因素（EFS：良好反应者为64%，不良反应者为50%）。
尽管根据风险调整了治疗强度，但肿瘤体积仍保留其预后意义；然而，切点向更大体积偏移。
