Jürgens H, Exner U, Gadner H, Harms D, Michaelis J, Sauer R, Treuner J, Voûte T, Winkelmann W, Winkler K
University Children's Hospital Düsseldorf, West Germany.
Cancer. 1988 Jan 1;61(1):23-32. doi: 10.1002/1097-0142(19880101)61:1<23::aid-cncr2820610106>3.0.co;2-m.
The German Society of Pediatric Oncology in 1981 initiated the Cooperative Ewing's Sarcoma Study (CESS 81) using a four-drug combination of chemotherapy prior to definitive local control with surgery and/or radiation. From January 1, 1981 until February 28, 1985, 93 patients were registered at the trial office from 54 participating institutions in West Germany, Austria, Switzerland, and the Netherlands. On February 1, 1987, 54 of 93 patients were disease-free. Using the Kaplan-Meier life table analysis, the estimated disease-free survival (DFS) rate was 60% at 36 months and 55% at 69 months. The median period of observation was 29 months, ranging from 22 months to 69 months. Twenty-one of 93 patients (23%) had local failure, 18 of 93 patients (19%) developed systemic metastases. The local failure rate was particularly high in patients treated with radiation and was reduced when radiation planning was centralized within the study based upon the extent of disease at diagnosis. Cox regression analysis of prognostic factors showed that tumor volume was a significant factor influencing prognosis. The estimated 3-year DFS rate was 80% for patients with small tumors (volume less than 100 ml) compared to 31% for patients with large tumors (volume greater than or equal to 100 ml). In patients who had surgery for local control, the histologic response to chemotherapy was analyzed on the surgical specimen and had a strong influence on survival: 79% DFS at 3 years for patients with less than 10% viable tumor (good responders) compared to 31% DFS for patients with more than 10% viable tumor (poor responders). Tumor load and responsiveness to chemotherapy are the two major factors influencing prognosis in patients with primary Ewing's sarcoma of bone.
1981年，德国小儿肿瘤学会启动了尤因肉瘤合作研究（CESS 81），在通过手术和/或放疗进行确定性局部控制之前，使用四种药物联合化疗。从1981年1月1日至1985年2月28日，来自西德、奥地利、瑞士和荷兰的54个参与机构的93名患者在试验办公室登记。1987年2月1日，93名患者中有54名无病。使用Kaplan-Meier生存表分析，估计无病生存率（DFS）在36个月时为60%，在69个月时为55%。中位观察期为29个月，范围从22个月至69个月。93名患者中有21名（23%）出现局部失败，93名患者中有18名（19%）发生全身转移。接受放疗的患者局部失败率特别高，当根据诊断时疾病范围在研究中集中放疗计划时，该率降低。预后因素的Cox回归分析表明，肿瘤体积是影响预后的重要因素。小肿瘤（体积小于100 ml）患者的估计3年DFS率为80%，而大肿瘤（体积大于或等于100 ml）患者为31%。在接受手术进行局部控制的患者中，对手术标本分析了化疗的组织学反应，其对生存有强烈影响：存活肿瘤少于10%的患者（反应良好者）3年DFS率为79%，而存活肿瘤超过10%的患者（反应不良者）为31%。肿瘤负荷和对化疗的反应性是影响原发性骨尤因肉瘤患者预后的两个主要因素。
