中文摘要：

为研究乳头状肾癌的临床病理特征、鉴别诊断与预后，对11例乳头状肾癌进行形态学观察、免疫组化染色与TNM分期，并随访患者。结果发现：11例乳头状肾癌镜下以乳头状、乳头-管状结构为主，45．4％（5／11）为1型乳头状肾癌，乳头表面被覆小细胞，胞质稀少，单层排列；54．5％（6／11）为2型乳头状肾癌，肿瘤细胞核分级高，胞质嗜酸性，假复层排列。免疫组化，11例乳头状肾癌AMACR、CK、CD10、CD117、Vim、MDM2的表达率分别为100％、72．7％、36．4％、36．4％、36．4％，0％，1型乳头状肾癌CK7的阳性率为100％，2型乳头状肾癌中Topoisomerase Ⅱα为66．6％，P53阳性率为50％。乳头状肾癌具有特殊的临床病理特征，是一类独立的疾病单元，AMACR、CK7、TopⅡα表达有助于乳头状肾癌及亚型的诊断，CK7、TopⅡα与P53的表达可能与预后相关。

英文摘要：

Purpose To study the clinicopathologic features, differential diagnosis and prognosis of Papillary renal cell carcinoma （PRCC）. Methods The histopathologic findings and immunophenotype of eleven cases of Papillary renal cell carcinoma were studied. Follow-up data were also analyzed. Results PRCC was composed of papillary and tubulopapillary architecture,45.4% （5/11） are type 1 PRCC,coverd by small cells with scanty cytoplasm;54.5% （6/11） are type 2 PRCC, with higher nuclear grade, eosinophilic cytoplasm and pseudostratified nuclei on papillary cores. Immunohistochemical staining showed that in 11 cases of PRCC AMACR, CK, CD10, CD117, Vim, MDM2 positively expressed in 100%, 72.7 %, 36.4 %, 36.4 %, 36.4%, 0%, respectively. All of type 1 PRCC express CK7 （ 100% ）, the positiverate of Topoisomerase Ⅱα（TopⅡα）,P53 in type 2 PRCC is 66.6% ,50% ,respectively. Conclusions PRCC has unique clinicopathological features, which is a distinct entity. The expressions of AMACR, CK7, Top Ⅱα are helpful in the distinction of PRCC and its subtype. The expression of CK7 ; Top Ⅱα and P53 may be related to prognosis of PRCC.