CAJ | 학술논문

背景和目的:尤文氏肉瘤家族肿瘤(Ewing's sarcoma family of tumor,ESFT)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 ESFT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ESFT.结果:骨尤文氏肉瘤(Ewing's sarcoma of bone,ETB)23例,骨外尤文氏肉瘤(extraosseous Ewing's sarcoma,EOE)21例,外周性原始神经外胚叶瘤(peripheral primitive neuroectodennal tumor,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(overall survival,OS)率分别为63%、20%,3年无事件生存(events-free survival,EFS)率分别为46%、18%,两组间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(x~2=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(x~2=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.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(χ~2=7.5919.212,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(χ~2=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.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(x~2=7.5919.212,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揹景和目的:尤文氏肉瘤傢族腫瘤(Ewing's sarcoma family of tumor,ESFT)噁性度高、進展快,其最佳治療方法目前仍在探討中.本研究旨在分析 ESFT的臨床特點和探討其治療方法.方法:迴顧性分析1995年1月至2008年4月中山大學腫瘤防治中心收治的92例初治ESFT.結果:骨尤文氏肉瘤(Ewing's sarcoma of bone,ETB)23例,骨外尤文氏肉瘤(extraosseous Ewing's sarcoma,EOE)21例,外週性原始神經外胚葉瘤(peripheral primitive neuroectodennal tumor,PNET)43例,Askin瘤5例.中位隨訪時間31.5箇月(10-137箇月).跼限期綜閤治療38例,單一治療19例.兩組3年生存(overall survival,OS)率分彆為63%、20%,3年無事件生存(events-free survival,EFS)率分彆為46%、18%,兩組間生存差異具有統計學意義(P均<0.001).跼限期綜閤治療患者在全身化療的基礎上加用手術加或不加放療組遠期生存均優于化療+放療組(x~2=7.591、9.212,P=0.006、0.002).CAV/IE交替方案對跼限期接受綜閤治療患者延長瞭無事件生存期,但其總生存時間差異無統計學意義(x~2=6.950、3.530,P=0.008、0.06).多因素生存分析顯示治療模式以及療效是獨立的預後因素.結論:綜閤治療能明顯改善跼限期ESFT患者療效和生存.手術加化療加或不加放療的治療模式在療效和生存方麵優于化療加放療治療模式.治療模式和近期療效是獨立的預後因素.閤治療患者在全身化療的基礎上加用手術加或不加放療組遠期生存均優于化療+放療組(χ~2=7.5919.212,P=0.006、0.002).CAV/IE交替方案對跼限期接受綜閤治療患者延長瞭無事件生存期,但其總生存時間差異無統計學意義(χ~2=6.950、3.530,P=0.008、0.06).多因素生存分析顯示治療模式以及療效是獨立的預後因素.結論:綜閤治療能明顯改善跼限期ESFT患者療效和生存.手術加化療加或不加放療的治療模式在療效和生存方麵優于化療加放療治療模式.治療模式和近期療效是獨立的預後因素.閤治療患者在全身化療的基礎上加用手術加或不加放療組遠期生存均優于化療+放療組(x~2=7.5919.212,P=0.006、0.002).CAV/IE交替方案對跼限期接受綜閤治療患者延長瞭無事件生存
배경화목적:우문씨육류가족종류(Ewing's sarcoma family of tumor,ESFT)악성도고、진전쾌,기최가치료방법목전잉재탐토중.본연구지재분석 ESFT적림상특점화탐토기치료방법.방법:회고성분석1995년1월지2008년4월중산대학종류방치중심수치적92례초치ESFT.결과:골우문씨육류(Ewing's sarcoma of bone,ETB)23례,골외우문씨육류(extraosseous Ewing's sarcoma,EOE)21례,외주성원시신경외배협류(peripheral primitive neuroectodennal tumor,PNET)43례,Askin류5례.중위수방시간31.5개월(10-137개월).국한기종합치료38례,단일치료19례.량조3년생존(overall survival,OS)솔분별위63%、20%,3년무사건생존(events-free survival,EFS)솔분별위46%、18%,량조간생존차이구유통계학의의(P균<0.001).국한기종합치료환자재전신화료적기출상가용수술가혹불가방료조원기생존균우우화료+방료조(x~2=7.591、9.212,P=0.006、0.002).CAV/IE교체방안대국한기접수종합치료환자연장료무사건생존기,단기총생존시간차이무통계학의의(x~2=6.950、3.530,P=0.008、0.06).다인소생존분석현시치료모식이급료효시독립적예후인소.결론:종합치료능명현개선국한기ESFT환자료효화생존.수술가화료가혹불가방료적치료모식재료효화생존방면우우화료가방료치료모식.치료모식화근기료효시독립적예후인소.합치료환자재전신화료적기출상가용수술가혹불가방료조원기생존균우우화료+방료조(χ~2=7.5919.212,P=0.006、0.002).CAV/IE교체방안대국한기접수종합치료환자연장료무사건생존기,단기총생존시간차이무통계학의의(χ~2=6.950、3.530,P=0.008、0.06).다인소생존분석현시치료모식이급료효시독립적예후인소.결론:종합치료능명현개선국한기ESFT환자료효화생존.수술가화료가혹불가방료적치료모식재료효화생존방면우우화료가방료치료모식.치료모식화근기료효시독립적예후인소.합치료환자재전신화료적기출상가용수술가혹불가방료조원기생존균우우화료+방료조(x~2=7.5919.212,P=0.006、0.002).CAV/IE교체방안대국한기접수종합치료환자연장료무사건생존
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 clinicaI characteristjcs 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,10-137months).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(χ~2=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(χ~2=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.