Peng Rou-Jun, Sun Xiao-Fei, Xiang Xiao-Juan, Zhen Zi-Jun, Ling Jia-Yu, Tong Gang-Ling, Xia Yi, Xu Guang-Chuang, Jiang Wen-Qi
State Key Laboratory of Oncology in South China, Guangzhou, Guangdong, 510060,P. R. China.
Ai Zheng. 2009 Dec;28(12):1304-9. doi: 10.5732/cjc.008.10609.
Ewing's sarcoma family of tumor (ESFT) is aggressive. The optimal therapy modality for ESFT is still to be found. This study was to explore the clinical characteristics and therapy for ESFT.
Ninety-two cases of ESFT were collected from January 1995 to April 2008 in Sun Yat-sen University Cancer Center and analyzed retrospectively.
Of 92 cases, 23 were Ewing's sarcoma of bone, 21 extraosseous Ewing's sarcoma, 43 peripheral primitive neuroectodermal tumor, and 5 Askin tumor. Median follow-up time was 31.5 months (range, 10-137 months). Thirty-eight patients received multidisciplinary therapy and 19 single model therapy in non-metastasis group. Three-year overall survival (OS) and event-free survival (EFS) were significantly different between non-metastatic multidisciplinary therapy group and non-metastatic single model group (63% vs. 20%, 46% vs. 18%, respectively, P<0.001). The patients who received surgery plus chemotherapy and plus radiation or not had longer survival than those treated with chemotherapy plus radiation in non-metastatic multidisciplinary therapy group (Chi2=7.591, 9.212; P=0.006, 0.002). CAV/IE alternative regimen was superior to other regimens in event-free survival, but not in overall survival (Chi2=6.950, 3.530; P=0.008, 0.06). Cox regression analysis suggested therapy model and response to treatment were independent prognostic factors for ESFT.
Our studying showed multidisciplinary therapy could significantly improve non-metastatic ESFT patients' survival. Chemotherapy plus surgery and plus radiation or not were superior to chemotherapy plus radiation in local control for the non-metastatic ESFT. Therapy model and response were independent prognostic factors.
尤因肉瘤家族性肿瘤（ESFT）具有侵袭性。ESFT的最佳治疗方式仍有待探索。本研究旨在探讨ESFT的临床特征及治疗方法。
回顾性分析1995年1月至2008年4月在中山大学肿瘤防治中心收集的92例ESFT病例。
92例中，骨尤因肉瘤23例，骨外尤因肉瘤21例，外周原始神经外胚层肿瘤43例，Askin瘤5例。中位随访时间为31.5个月（范围10 - 137个月）。非转移组中38例患者接受多学科治疗，19例接受单一模式治疗。非转移多学科治疗组与非转移单一模式组的三年总生存率（OS）和无事件生存率（EFS）有显著差异（分别为63%对20%，46%对18%，P<0.001）。在非转移多学科治疗组中，接受手术加化疗及是否加放疗的患者比接受化疗加放疗的患者生存期更长（χ2 = 7.591，9.212；P = 0.006，0.002）。CAV/IE交替方案在无事件生存率方面优于其他方案，但在总生存率方面并非如此（χ2 = 6.950，3.530；P = 0.008，0.06）。Cox回归分析表明治疗模式和对治疗的反应是ESFT的独立预后因素。
我们的研究表明多学科治疗可显著提高非转移ESFT患者的生存率。对于非转移ESFT，化疗加手术及是否加放疗在局部控制方面优于化疗加放疗。治疗模式和反应是独立的预后因素。
