Abstract: Objective To study the clinicopathologlc features, immunophenotype and differential diagnosis of cystic disease of kidney. Methods 3 cases of cystic diseases were observed under light microscope, HE and immunohistochemical staining. And the literature was reviewed. Results A11 of the three patients presented with loin pain and hematuria．Two cases of cystic nephroma (CN) and one cases of multilocular cystic renal cell carcinoma were identified by pathological method. Cystic nephroma consisted of multilocular cysts and were lined by single epithelial cells. Immunohistochemically, the epithelial cell were positive for cytokeratin and EMA, and the stromal cells were positive for vimentin. Multilocular cystic renal cell carcinoma were lined by single to multilayered epithelial cells with clear cytoplasm. There existed aggressive clear cells between the cysts, which positive for cytokeratin and EMA, and negative for vimentin immunohistochemically. Conclusions Both CN and MCRCC are uncommon renal tumor． There are similar symptoms and images between these two diseases. Pathology is the key to diagnosis. Both of them have good prognosis.

摘要： 目的 探讨肾囊性病变的临床病理学特点、免疫表型和鉴别诊断。方法 采用常规HE和免疫组织化学染色，观察3例肾囊性病变组织学形态，并复习有关文献。结果 3个病例临床症状均为腰痛或血尿，最后经病理确诊为囊性肾瘤2例和多房性囊性肾癌1例。囊性肾瘤为多房囊腔组织，囊壁薄，内衬单层上皮。免疫表型：上皮细胞角蛋白（CK）、上皮细胞膜抗原(EMA)均呈阳性表达；间质成分波形蛋白阳性。多房性囊性肾癌囊壁被覆单层或多层胞浆透明的细胞，间隔内含聚集的透明细胞巢。免疫表型：透明细胞CK和EMA阳性，CD68阴性。结论 囊性肾瘤和多房性囊性肾癌均是少见的肾脏肿瘤。两种病变临床症状和影像学改变极为相似，确诊需依赖病理检查。两种病变均预后良好。

关键词: 囊性肾瘤, 多房性囊性肾癌, 免疫组织化学