目的：探讨透明细胞(管状)乳头状肾细胞癌(clear cell tubulopapillary renal cell carcinoma，CCTPRCC) 临床病理学特征。方法：复习2012年至2014年肾细胞癌病理学切片，筛选CCTP-RCC病 例。观察CCTP-RCC巨检及镜检特征，行免疫组化染色以及分子遗传学检测。结果：肾细胞癌中 CCTP-RCC占2.0%(4/201)。肿瘤平均直径2.8 cm(范围1.2~4.5 cm)。囊性、乳头状、管状以及实性 生长方式出现频率分别是100%(4/4)、100%(4/4)、100%(4/4)以及75%(3/4)。4例(100%)细胞轻度 异型性。Fuhrman细胞核分级2级3例(75%)，1级1例(25%)。细胞核远离基底膜的细胞占17.5%(范 围10%~20%)，其他位置占82.5%(范围80%~90%)。4例(100%)见平滑肌组织，3例(75%)见发育不全 血管。3例(75%)T1a，1例(25%)T1b，所有病例均为I期肿瘤。免疫组化CK7 4例(100%)弥漫强阳 性；CD10 3例(75%)阴性反应，1例(25%)局灶弱阳性。分子遗传学4例FISH检测17号染色体为二倍 体。平均随访时间15.5(3~23)个月，所有病例没有局部复发、淋巴结转移以及远处转移。结论： CCTP-RCC是一种少见的RCC亚型；病理诊断应结合生长方式、细胞学以及间质表现三方面综合 分析；典型免疫表型是CK7弥漫强阳性，CD10阴性或局灶阳性。
Clinicopathological analysis of clear cell tubulopapillary renal cell carcinoma
Objective: The aim of the present study was to investigate the clinicopathological features of clear cell tubulopapillary renal cell carcinoma (CCTP-RCC). Methods: we reviewed the pathological slices of renal cell carcinoma cases during 2012 to 2014, and the cases diagnosed as CCTP-RCC were enrolled in our study. We observed macroscopic and microscopic characteristics of the slices, and carried out immunohistochemistry (IHC) and molecular genetics test. Results: CCTP-RCC accounted for 2.0% (4/201) in renal cell carcinoma. The average diameter of tumors was 2.0 cm (range from 1.2 to 4.5 cm). The frequencies of growth pattern of cystic, papillary, tubular and solid tumors were 100% (4/4), 100% (4/4), 100% (4/4) and 75% (3/4), respectively. A total of 4 (100%) cases were mild atypical cells. Three (75%) cases were in grade 2 and 1 (25%) case was in grade 1 in Fuhrman nuclear grade. The cells with nucleus away from the basement membrane accounted for 17.5% (range from 10% to 20%), and other locations accounted for 82.5% (from 80% to 90%). The smooth muscle was observed in all cases, and hypoplastic vascular was observed in 3 of 4 cases (75%). Of the 4 cases, 3 (75%) cases were in T1a, 1 (25%) case was in T1b. All cases were stage I tumors. CK7 was diffusely strong positive in all cases, and CD10 was negative in 3 (75%) cases, and focal weakly positive in 1 (25%) case from the IHC results. The diploid chromosome 17 was detected in 4 cases by FISH test. The mean follow-up time was 15.5 months (range from 3 to 23 months). The local relapse, lymph node and distant metastasis were not observed in all patients with CCTP-RCC. Conclusion: CCTP-RCC is a rare subtype of RCC. As for pathological diagnosis, growth pattern, cytology and interstitial performance should be analyzed synthetically. The typical immune phenotype of CCTPRCC shows that CK7 is diffuse strong positive and CD10 is negative or focal weakly positive.