摘要目的：探讨透明细胞乳头状肾细胞癌（ CCPRCC）的临床和病理特征，旨在提高对该类肿瘤的认识。方法回顾性分析6例典型病例的临床资料、组织形态学和免疫表型，随访患者及复习相关文献。结果男3例，女3例，平均发病年龄56岁。肿块最大径1.0～4.5 cm，呈实性或囊实性。镜下观察，肿瘤具有厚的包膜，瘤细胞有多种排列方式：管状、乳头状、分支管状、腺泡状、巢状、缎带状、囊状或微囊状等，腔内或囊内常见分泌性蛋白液聚集。瘤细胞小至中等大，立方形，胞质丰富、透明，可见特征性类似于分泌早期子宫内膜核下空泡。瘤细胞核呈圆形或椭圆形，Fuhrman核级1级或2级，核分裂象罕见。肿瘤间质及纤维血管轴心内均无泡沫样组织细胞及含铁血黄素沉积，无沙砾体形成。2例间质内出现局灶或广泛性的血管平滑肌瘤样／平滑肌瘤样成分。6例肿瘤均未见肾窦侵犯、血管累犯、肿瘤性坏死。免疫组织化学示6例瘤细胞均弥漫强表达CK7，表达CA9（特征性的“杯状”着色模式）、CK （ AE1／AE3）、波形蛋白、CK8、CK18和 CK19；不同程度表达上皮细胞膜抗原（EMA）、PAX-8；Ki-67阳性表达约≤5％；6例均不表达CD10、P504S、CD117、TFE3和TFEB。术后随访时间8～27个月，平均14个月，均无复发和转移。结论 CCPRCC是一种低度恶性的肾细胞癌，具有相对特征性的组织学形态和免疫表型，诊断时应与透明细胞肾细胞癌和乳头状肾细胞癌相鉴别。

abstractsObjective To study the clinicopathologic features of clear cell papillary renal cell carcinoma ( CCPRCC ) .Methods The clinical, morphologic and immunohistochemical characteristics of 6 cases of CCPRCC were reviewed, with analysis of follow-up data.Results There were altogether 3 men and 3 women.The mean age of patients was 56 years.The size of tumors ranged from 1.0 to 4.5 cm in greatest dimension.They had solid or solid-cystic cut surface.Histologically, the tumors were encapsulated and showed several morphologic patterns, with tubules, papillae, acini, interconnecting ribbons and macro/microcysts lined by single layer of cells with clear or small amount of eosinophilic cytoplasm and low-grade nuclei (corresponding to Fuhrman grade 1 or 2).Mitotic figures were rarely seen.Characteristically, there was linear arrangement of the nuclei away from the basement membrane, conferring an appearance similar to that of endometrial glands in early secretory phase.Tubules and cysts contained serosanguineous fluid or colloid-like secretion were identified.No foamy histiocytes, psammomatous calcifications or hemosiderin was present in the papillary areas.Two of the tumors showed focal or extensive angioleiomyoma/leiomyoma-like components.No coagulative necrosis, sarcomatoid dedifferentiation, nor microscopic vascular invasion was observed. Immunohistochemically, all tumors showed strong co-expression of CK7 and CA9 ( with characteristic“goblet” staining pattern).The staining for EMA, CK (AE1/AE3), vimentin, CK8, CK18, CK19 and PAX-8 were also positive in all cases.Ki-67 was expressed in less than or about 5%of the tumor cell nuclei.The staining for CD10, P504S, CD117, TFE3 and TFEB was negative.Follow-up data were available in all patients, with mean duration of 14 months ( range=7 to 27 months) .All of the patients were disease-free after operation.Conclusion CCPRCC is a special type of low-grade renal neoplasm with characteristic histopathologic and immunohistochemical features.It needs to be distinguished from clear cell renal cell carcinoma or papillary renal cell carcinoma.