腹膜后纤维瘤病1例报告并文献复习  

Retroperitoneal fibromatosis: 1 case report with literature review

作　　者：代海涛[1] 刘昌茂[1] 夏安[1] 熊鹰[1] 呙林杰[1] 刘远华[1] 郑江[1] 陈益[1] 

机构地区：[1]长江大学附属第一医院泌尿外科,湖北荆州434000

出　　处：《现代肿瘤医学》2015年第22期3289-3291,共3页Journal of Modern Oncology

摘　　要：目的:探讨腹膜后纤维瘤病临床特点。方法:报道1例腹膜后纤维瘤病侵袭输尿管致严重血尿的诊治,并复习有关文献。结果:术中发现腹膜后右侧输尿管末端有一直径6cm实性肿瘤,固定于盆壁,与髂外动静脉致密粘连,包裹右侧输尿管末端,上段输尿管扩张。行肿瘤大部切除术+输尿管再植术,切除肿瘤大部分为肌瘤样组织,中间坏死形成囊腔,与输尿管相通;术中快速冰冻片报告梭形细胞肿瘤,良恶性待定;术后病理报告为腹膜后纤维瘤病,免疫组化:CD34(-)、CD117(-)、Dog-1(-)、SMAS-100(-)、Vimentin(+)、Desmin(-)、NSE(-)。结论:腹膜后纤维瘤病是一种十分罕见的软组织肿瘤,可以因发生位置不同而以不同临床症状就诊,提高对这一肿瘤的认识可以减少误诊。Objective： To investigate the clinical features of retroperitoneal fibromatosis. Methods： Describing and reporting the diagnosis and treatment of a case of retroperitoneal fibromatosis,which was invasive to the right ureter,caused severe hematuria. The literature had been reviewed and discussed. Results： There was a 6cm solid tumor,fixed on the pelvic wall,external iliac vein with dense adhesions,parcel right ureter end,with upper ureteral dilatation. We underwent tumor subtotal ＋ ureteral reimplantation. Most of the tumor were fibroid tissue,necrosis intermediate formation of cysts,which interlinked ureter. Intraoperative frozen section slice reported spindle cell tumors. Postoperative pathology reported retroperitoneal fibromatosis. Immunohistochemistry： CD34（-）,CD117（-）,Dog-1（-）,SMAS-100（-）,Vimentin（ ＋）,Desmin（-）,NSE（-）. Conclusion： Retroperitoneal fibromatosis is a very rare soft tissue tumor which can occur different clinical symptoms. To improve the understanding of this tumor can reduce misdiagnosis.

关 键 词：纤维瘤病 腹膜后 免疫组化 

分 类 号：R735.5[医药卫生—肿瘤]