Department of Pediatric Hematology and Oncology, University Hospital Münster, Münster, Germany.
Pediatr Blood Cancer. 2011 Oct;57(4):549-53. doi: 10.1002/pbc.23040. Epub 2011 Mar 25.
The prognosis in patients with relapsed Ewing sarcoma is unfavorable. Our investigation identifies factors predicting for the outcome following relapse.
We analyzed type of relapse, time to relapse and overall survival after relapse (OSr) in 714 patients with first recurrence. All patients had been treated within the Cooperative Ewing Sarcoma Studies (CESS) 81 or 86, or the European Intergroup CESS (EICESS 92). OSr time was calculated from diagnosis of first relapse to last follow-up or death.
Median follow-up time from diagnosis of primary disease was 2.2 years (mean = 4.0; range: 0.2-24.9). Relapse sites were local in 15%, combined local and systemic in 12%, and systemic in 73%. Among patients with a localized primary tumor, 20% relapsed locally, while 12% showed combined and 68% systemic relapse. When the primary disease was disseminated, 82% developed systemic, 13% combined, and 5% local relapse. Five-year OSr was 0.13 (SE = 0.01). Outcome following local relapse, with a 5-year survival rate of 0.24 (P < 0.001), was superior to outcome after systemic or combined recurrence. Five-year OSr was 0.07 (SE = 0.01) in patients who relapsed 0-2 years after the diagnosis of primary disease, as compared to a 5-year OSr of 0.29 (SE = 0.03) when relapse occurred later.
5-year OSr in Ewing sarcoma is poor (<0.2). Prognostically favorable factors are: late onset (>2 years) and strictly localized relapse.
复发性尤因肉瘤患者的预后不佳。我们的研究确定了复发后预测结果的因素。
我们分析了 714 例首次复发患者的复发类型、复发时间和复发后总生存期（OSr）。所有患者均在 CESS81 或 86 或欧洲协作组 Ewing 肉瘤研究（EICESS92）中接受治疗。OSr 时间从首次复发的诊断到最后随访或死亡计算。
从原发疾病诊断到中位随访时间为 2.2 年（平均值=4.0；范围：0.2-24.9）。复发部位为局部 15%，局部合并全身 12%，全身 73%。在局部原发性肿瘤患者中，20%局部复发，12%出现局部合并全身复发，68%出现全身复发。当原发疾病播散时，82%发生全身复发，13%发生局部合并全身复发，5%发生局部复发。5 年 OSR 为 0.13（SE=0.01）。局部复发后 5 年生存率为 0.24（P<0.001），优于全身或局部合并复发后的生存率。诊断后 0-2 年复发的患者 5 年 OSR 为 0.07（SE=0.01），而诊断后 2 年以上复发的患者 5 年 OSR 为 0.29（SE=0.03）。
尤因肉瘤 5 年 OSR 较差（<0.2）。预后良好的因素是：发病时间晚（>2 年）和严格局部复发。
