92例初治尤文氏肉瘤家族肿瘤综合治疗疗效和生存分析

背景和目的：尤文氏肉瘤家族肿瘤（Ewing’ssarcomafamilyoftumor，ESFF）恶性度高、进展快，其最佳治疗方法目前仍在探讨中。本研究旨在分析ESFF的临床特点和探讨其治疗方法。方法：回顾性分析1995年1月至2008年4月中山大学肿瘤防治中心收治的92例初治ESFF。结果：骨尤文氏肉瘤（Ewing’ssarcomaofbone，ETB）23例，骨外尤文氏肉瘤（extraosseousEwing’Ssarcoma，EOE）21例，外周性原始神经外胚叶瘤（peripheralprimitiveBeuroectodermaltumor，PNET）43例，Askin瘤5例。中位随访时间31．5个月（10～137个月）。局限期综合治疗38例，单一治疗19例，两组3年生存（overallsurvival，OS）率分别为63％、20％，3年无事件生存（events．freesurvival，EFS）率分别为46％、18％，两组问生存差异具有统计学意义（P均〈0．001）。局限期综合治疗患者在全身化疗的基础上加用手术加或不加放疗组远期生存均优于化疗＋放疗组（x2=7．591、9．212，P=0．006、0．002）。CAV／IE交替方案对局限期接受综合治疗患者延长了无事件生存期，但其总生存时间差异无统计学意义（X2=6．950、3．530，P=0．008、0．06）。多因素生存分析显示治疗模式以及疗效是独立的预后因素。结论：综合治疗能明显改善局限期ESFT患者疗效和生存．手术加化疗加或不加放疗的治疗模式在疗效和生存方面优于化疗加放疗治疗模式。治疗模式和近期疗效是独立的预后因素。

Efficacy and survival of 92 cases of Ewing s sarcoma family of tumor initially treated with multidisciplinary therapy

Background and Objective： 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. Methods： Ninety-two cases of ESFT were collected from January 1995 to April 2008 in Sun Yat-sen University Cancer Center and analyzed retrospectively. Result= 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 （X2=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 （X2=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. Conclusions： 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.