中文摘要：

目的：阐明肾脏肌纤维瘤临床病理特点。方法：回顾性分析1例肾脏肌纤维瘤的临床表现、组织学和免疫组织化学特点。结果：患者主要临床表现为腰痛,CT检查发现左肾中下极巨大囊实性占位。大体检查：肾中下极见一灰白结节,体积10cm×7cm×5cm,切面灰白质韧,与周围组织界限较清,其间可见多个小囊腔及结节,直径均小于1cm。光镜观察：低倍镜下,肿瘤呈多结节状增生,结节周边部分一般为梭形肌样细胞排列成短束状或旋涡状结构,瘤细胞胞浆丰富,染色淡,细胞核狭长;结节中心部位瘤细胞圆形、短梭形、多角形,胞浆少,胞核深染,沿薄壁的＂鹿角状＂分支的小血管排列,呈所谓的血管外周细胞瘤样结构,局部间质可见玻璃样变性。部分区域两种类型细胞随意分布。免疫组化染色结果 Vimentin弥漫阳性,SMA、Desmin、Bcl-2、HHF-35灶性阳性,WT-1、EMA、AE1/AE3、HMB45、CD34、S-100、CD117、CD10、ALK均阴性,Ki67阳性细胞指数约为2%。结论：肾脏肌纤维瘤是一种罕见的良性肿瘤,明确诊断必须借助于临床及组织学改变和免疫组织化学特征检查,首选治疗是肿瘤全切手术。

英文摘要：

Objective：To investigate the clinical and pathological characteristics of myofibroma of the kidney（MK）.Methods： Clinical manifestations,histological and immunohistochemical features of 1 MK patient was analyzed retrospectively.Results： The main clinical manifestations of MK patient was lumbago,a huge solid and cystic occupying lesion was found in the mid-lower pole of left kidney by CT.Specimen examination after surgery found that a gray nodule was at the lower pole of the kidney with the size of 10cm×7cm×5cm,gray section and tenacious texture.The boundary between the nodule and surrounding tissue was clear and many small cysta and nodules with the diameter less than 1cm were found.The microscopic examination has found： At low power,there were a multinodular proliferation,usually within the periphery of the nodules,there were spindleshaped muscle appearance cells with abundant cytoplasm,lighted staining,long oval nuclei and arranged in short fascicles or whorls.Within the centre of the nodules,were round,short spindleshaped,polygonal and stained nuclei,the cells arranged along the thin-walled small vasules like staghorn which was called hemangiopericytoma-like vascular pattern.Hyaline degeneration can be found in some interstitial substance.Occasional cases have a more random distribution of the two cell types and in some cases.Immunohistochemical results showed the tumor cells were positive for vimentin,focally positive for desmin,SMA,Bcl-2 and HHF-35 but negative for WT-1,EMA,AE1/AE3,HMB45,CD34,S-100,CD117,CD10,ALK.Conclusion： Myofibroma of the kidney is a rare tumor and diagnosed correctly by its characters of clinic,histopathlogy and immunohistochemistry.The first choice of treatment is total tumor resection.