摘要目的：提高对原发性肾滑膜肉瘤的诊治水平。方法回顾性分析1例原发性肾滑膜肉瘤患者的资料，女，54岁。因左腰部疼痛6 d于2012年2月14日入院。增强CT检查示左肾上极一直径约2．0 cm的类圆形低高混杂密度影，内见点状钙化影，左肾周见低高混杂密度影，考虑左肾肿瘤破裂出血、肾周血肿积液。全麻复合硬膜外麻醉下行左肾探查术，术中见左肾外侧肾周筋膜内有巨大血肿，与周围脏器粘连，切开血肿后吸出陈旧出血及血块约1000 ml，内混杂鱼肉样组织，收集组织行快速病理检查，结果为肉瘤样组织，遂行根治性肾切除术，手术顺利。结果术后病理报告：左肾肿瘤体积3．0 cm×2．5 cm×1．5 cm，灰白色鱼肉样改变并多处出血及坏死，瘤细胞呈梭形，细胞界限欠清，呈束状排列，核呈卵圆形或杆状，有大量核分裂象。免疫组化染色检查：BCL-2（＋），Vimentin （＋），EMA（＋），CD79a（＋），SMA灶性（＋），CD34（-）， CK（-）， CD10（-）。病理诊断为（左肾）肉瘤，符合滑膜肉瘤，（左肾上极）出血性囊肿。术后口服舒尼替尼37．5 mg，1次/d治疗，随访6个月未见肿瘤复发及转移。结论原发性肾滑膜肉瘤罕见，临床症状及影像学检查诊断困难，确诊靠病理或遗传分子学检查。治疗首选根治性肾切除术并药物辅助治疗，预后较差，采用舒尼替尼治疗可能获得一定疗效。

abstractsObjective To improve the diagnosis and therapy of primary renal synovial sarcoma . Methods A single case of primary renal synovial sarcoma was reported .A 54-year-old female presented with left flank pain for six days .Enhanced CT scan revealed a nearly-circular mass with the diameter of 2.0 cm in the superior portion of the left kidney .It showed low and high density with punctate calcification .There were low and high density in left kidney around .A possible diagnosis of left renal tumor with rupturing was given prior to exploratory operation .During the operation , a large hematoma was found on the lateral peri-re-nal fascia, which adjoined with other organs .The hematoma was opened and the 1 000 ml dated clot and blood were removed .The tissue inside the hematoma showed fish meat like .The fast pathologic examination showed sarcomatoid tissue , then the case underwent radical left nephrectomy . Results Gross pathologic examination showed a ashen and fish-liked tumor of 3.0 cm×2.5 cm×1.5 cm with multiple sites of necrosis and hemorrhage .The histopathological examination revealed the tumor cells were spindle shaped .The tumor cells organized as bundle with indistinct border line and larger quantity of mitotic phase , which were positive for BCL-2, vimentin, EMA, CD79a and SMA, but negative for CD34, CK and CD10.A diagnosis of renal syn-ovial sarcoma was then determined .Targeted therapy with sunitinib ( 37.5 mg po qd ) was carried out postopreratively .There was no recurrence and metastasis during the six months following up . Conclusions Primary renal synovial sarcoma is an extremely rare entity .It is very difficult to diagnose through clinical symptoms and imaging .The accurate diagnosis is based on pathology or genetic and molecular tests .Radical nephrectomy and adjuvant therapy are the preferred strategy for the treatment .The prognosis is poor .Targeted therapy with sunitinib may be effective .