腹膜后纤维瘤病1例报告并文献复习

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。

Retroperitoneal fibromatosis:1 case report with literature review

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.