转移性肾滑膜肉瘤1例报告并文献复习  

Metastatic renal synovial sarcoma:a case report and literature review

作　　者：杨宪法[1] 赵耀瑞[2] 王勇[1] 杨福江[1] 郜海涛[1] 李航[1] 孙光[1] 徐勇[1] 

机构地区：[1]天津医科大学第二医院泌尿外科,天津市泌尿外科研究所,天津300211 [2]中国人民武装警察部队后勤学院附属医院泌尿外科

出　　处：《临床泌尿外科杂志》2015年第2期136-140,共5页Journal of Clinical Urology

摘　　要：目的:探讨肾脏滑膜肉瘤的临床表现、病理特点、诊断、鉴别诊断及治疗方法。方法:结合相关文献,回顾性分析1例转移性肾脏滑膜肉瘤患者的资料,女,49岁,因突发右腰腹部剧烈疼痛18h入院。B超及CT检查发现右肾上极囊实性占位,直径约12cm,考虑右肾肿瘤破裂出血,肾动脉栓塞后在全麻下行经腹腔根治性右肾切除术,术中见右肾上极肿物与周围脏器粘连,右肾静脉未见瘤栓,切除整个右肾及肿物。结果:肿物标本大体观:肾门偏上可见一12.8cm×11.2cm囊壁,囊内充满灰白色、鱼肉状组织;光镜下肿瘤由丰富的单向梭形细胞组成,未见上皮分化成份,肿瘤细胞成束状或片状排列,瘤细胞中等大小,较肥胖,细胞界线不清,核呈卵圆形或杆状,可见核仁,有大量核分裂象,伴发大量出血坏死;免疫组化:EMA(+),Vimentin(+),Inhinbin-α(+),CD99(+),Bcl-2(+);CK、CGA、Nse、Desmin、CD117、CD34均阴性。支持肾脏滑膜肉瘤。结合患者滑膜肉瘤多次手术史及曾通过RT-PCR方法检测出SYT-SSX融合基因mRNA表达,诊断为转移性肾滑膜肉瘤。未行进一步治疗,术后2个月死于癌转移。结论:转移性肾脏滑膜肉瘤极为罕见,高度恶性,预后极差。术前确诊困难,确诊主要依靠既往病史,病理检查,免疫组化染色,必要时可行分子生物学诊断。治疗上首选外科手术,术后需辅助进一步化疗或放疗。Objective：To investigate the clinical manifestations,pathologic features,diagnosis,differential diagnosis and treatment of the renal synovial sarcoma.Method：Combined with the relevant literature,we retrospectively analyzed the clinical data of a case of metastatic renal synovial sarcoma.A 49-year-old female presented with an 18-hour history of right flank pain and abdominal distension before admission.Ultrasound and CT revealed a12-cm diameter cystic and solid mass originating from the upper pole of the right kidney.A possible diagnosis of right renal tumor with rupturing was given prior to exploratory operation.The patient subsequently underwent radical right kidney resection by abdominal access under the general anesthesia after renal artery embolization.During the operation,the mass was found adhere to surrounding organs,and no tumor thrombus was in the right renal vein.The entire right kidney and tumor were resected.Result：Pathological examination showed there was a12.8cm ×11.2cm capsule wall at the upper pole of the renal hilum.The capsule filled with grey-white and fishshaped tissue.In the HE section,the tumor was composed of abundant unidirectional spindle cells without epithelial differentiation ingredients.The tumor cells organized as bundle or flake,which were medium-sized and polysarcia.The cell boundaries were unclear,and the nuclear showed oval or rod in which nucleoli were visible.There were a large quantity of mitotic phases with multiple sites of necrosis and hemorrhage.Immunohistochemical staining showed that the tumor cells were positive for the markers EMA,Vimentin,Inhinbin-a,CD99 and Bcl-2,but negative for CK,CGA,Nse,Desmin,CD117 and CD34.It confirmed the diagnosis of synovial sarcoma of the kidney.Combined with synovial sarcoma in patients with a history of multiple surgeries and the result of SYTSSX fusion gene mRNA expression by RT-PCR detection,the patient was diagnosed with metastatic renal synovial sarcoma.The patient received no further treatment and died of tumor metastasis two

关 键 词：肾肿瘤 滑膜肉瘤 转移 诊断 治疗 

分 类 号：R738.5[医药卫生—肿瘤] R737.11[医药卫生—临床医学]