肾集合管癌合并肾囊性透明细胞癌1例报告及文献复习

Synchronus renal collecting duct carcinoma and renal cystic clear cell carcinoma:Report of a case and review of literature

目的:探讨肾集合管癌(CDC)合并肾囊性透明细胞癌的临床病理特征及其诊断、鉴别诊断方法。方法:分析1例CDC合并肾囊性透明细胞癌患者的临床表现、组织形态学和免疫表型特征,并复习相关文献。结果:患者,男性,70岁,临床表现为无痛性血尿。影像学检查提示左肾占位。组织学示肾上极的肿瘤组织排列成不规则腺管状、乳头状,部分肿瘤细胞呈靴钉状突向腺腔内,间质纤维结缔组织明显增生,大量淋巴细胞浸润,肿瘤周围部分集合管腺上皮见异型增生。瘤细胞阳性表达波形蛋白(vimentin)、角蛋白7(CK7)、角蛋白19(CK19)、上皮膜抗原(EMA)、转录因子E3(TFE3)和E-钙黏素(E-cad),而角蛋白20(CK20)、CD31、CD34、CD10、CD117、肾脏特异性钙黏蛋白(ksp-cad)和α-甲基酰基辅酶A消旋酶(AMACR)表达则呈阴性。肾中部囊性肿瘤组织学表现为典型的肾囊性透明细胞癌,癌细胞阳性表达CD10、波形蛋白和CK19,而CK7、CD117表达则呈阴性。结论:CDC是一种少见的高度恶性肿瘤,其诊断依赖组织病理学和免疫组化标记。由于组织起源不同,CDC合并肾透明细胞癌的概率更小,但这种合并存在的情况仍有可能。

Objective To report a case of synchronous renal collecting duct carcinoma and renal cystic clear cell carcinoma, investigating its clinicopathological features, diagnosis and differential diagnosis. Methods The clinical presentations, histopathological features and immunophenotypic characteristics were studied in a case of renal collecting duct carcinoma coexisted with renal cystic clear cell carcinoma, and the related literatures were reviewed. Results A 70-year-old man complained of painless hematuria. A mass of left kidney was found by imaging examination. Histologically, tumor tissue in the upper portion of left kidney displayed irregular tubular and papillary patterns, and some of cells displayed hobnail-like cells with desmoplastic stroma reaction and a large number of lymphoid cells infiltration. Dysplasia was seen in collecting duct epithelium around the tumor. Immunohistochemically, the tumor cells were positive for vimentin, CK7, CK19, EMA, TFE3 and E-cad, but negative for CK20, CD31, CD34, CD10, CD117, ksp-cad and AMACR. In addition, a cystic mass was found in the medium portion of left kidney, which was a typical renal cystic clear cell carcinoma. The tumor cells were positive for CD10, vimentin and CK19,and negative for CK7 and CD117. Conclusious Collecting duct carcinoma of the kidney is a rare, highly aggressive malignant tumor and the diagnosis depends on its histopathologic and immunohistologic characteristics. The coexistence of renal collecting duct carcinoma and renal cystic clear cell carcinoma is rarer, but is possible.