摘要目的：探讨透明细胞乳头状肾细胞癌的临床病理特征、诊断、鉴别诊断。方法对11例透明细胞乳头状肾细胞癌进行光镜观察，行免疫组织化学检测、分子病理检测及随访。结果11例患者中女性3例，男性8例。年龄33～72岁，平均52畅5岁。肿块直径1～4 cm。光镜下肿瘤组织学可见以纤维血管间质为轴心的真性乳头状结构、管状腺泡状结构、微囊结构或实性区域等。在一个肿瘤中可以某一种结构为主，或多种结构相混合，但以广泛的真性乳头伴囊性结构为典型特点。肿瘤细胞均具有丰富透明的胞质，小到中等大小，立方形。核圆或椭圆形，具有轻度异型性，核分裂象罕见，大部分为Fuhrman 1级或2级。肿瘤间质未见出血及坏死。免疫表型：11例肿瘤组织均阳性表达角蛋白（CK）7、碳酸酐酶9（CAⅨ）、波形蛋白和缺氧诱导因子（HIF）-1α，8例表达 Ksp-cadherin， CD10、α-甲酰基辅酶A消旋酶（ P504S）和TFE3均为阴性。分子病理检测示11例肿瘤均未发现VHL基因突变及3p缺失。其中有3例肿瘤存在第3号染色体单倍体现象。8例患者获得随访，无肿瘤复发及转移，病情稳定。结论透明细胞乳头状肾细胞癌是一种低度恶性少见肿瘤，形态上需与多种具有透明细胞和乳头状结构的肾癌鉴别，这些肿瘤包括肾透明细胞癌、乳头状肾细胞癌和Xp11易位性肾癌。免疫组织化学和分子病理检测对它们的鉴别诊断有所帮助。

abstractsObjective To study the clinicopathological features , differential diagnosis and prognosis of clear cell papillary renal cell carcinoma ( CCPRCC ).Methods The histological , immunohistochemical , and molecular features were studied in 11 cases and follow-up data were also analyzed.Results There were a total of 3 females and 8 males.The age of patients were ranged from 33 to 72 years ( mean age 52.5 years).The diameters of tumors varied from 1cm to 4 cm.Histologically, papillary and cystic architecture were present at least focally in all tumors.The papillae were covered by small to medium-sized cuboidal cells with abundant clear cytoplasm and often showed extensive secondary branching , which were often folded and densely packed , resulting in a solid appearance.The nuclei were round and uniform in shape;nucleoli were not prominent ( Fuhrman grade 1 or 2 ).Neither mitotic figures nor necrosis was present.All 11 cases exhibited moderate to strong positivity for CK7, CA9, vimentin, and HIF-1α, coupled with negative reactions for CD10, P504S, and TFE3.Ksp-cadherin was positively expressed in 8 cases.VHL gene mutations were not found in all 11 cases.Losses of chromosomes 3 (monoploid chromosome 3) was detected in 3 cases.Conclusions CCPRCC is uncommon and seemed to be an indolent tumor.The differential diagnosis should be included tumors , which harbor clear cell and papillary structure including clear cell renal cell carcinoma , papillary renal cell carcinoma , Xp11 translocation renal cell carcinoma , and CCPRCC.Immunohistochemical and molecular analysis may be help for its diagnosis.