肾集合管癌的临床病理学分析  

Collecting duct carcinoma of the kidney:a clinicopathological analysis

作　　者：吴敏 张彦宁[1] 郑媛媛[1] 张淑红[1] 柳玮华[1] 陈光勇[1] WU Min;ZHANG Yan-ning;ZHENG Yuan-yuan;ZHANG Shu-hong;LIU Wei-hua;CHEN Guang-yong(Department of Pathology,Beijing Friendship Hospital,Capital Medical University,Beijing100050,China)

机构地区：[1]首都医科大学附属北京友谊医院病理科,北京100050

出　　处：《诊断病理学杂志》2023年第7期638-642,共5页Chinese Journal of Diagnostic Pathology

基　　金：国家重点研发计划“老年人多病共患临床大数据与生物样本库综合管理共享平台建设”(2020YFC2004800)项目。

摘　　要：目的探讨肾集合管癌(CDC)的临床病理特征、诊断、鉴别诊断及预后。方法回顾性分析8例CDC患者的临床病理特征、免疫组化结果,并复习相关文献。结果男性5例,女性3例,年龄38~78岁,平均年龄59岁;临床主要表现为肉眼血尿,发热,厌食乏力;大体检查肿瘤主要位于肾髓质,单发,最大径为3.8~6.3 cm,切面灰白色、质硬;镜下肿瘤细胞呈管状、管状乳头状、微乳头、实性及条索状排列,可见肉瘤样区域,间质促纤维组织增生,伴多量淋巴细胞、浆细胞及中性粒细胞浸润;细胞异型性明显,呈多边形或鞋钉状,核仁显著,核分裂象多见;免疫组化结果阳性表达情况为CK19(8/8)、CK7(8/8)、34βE12(7/8)、E-cadherin(7/8)和Vimentin(7/8),FH和SMARCB1(INI-1)均未缺失;随访3~57个月,4例于术后12个月内死亡,1例于术后36个月内死于肺纤维化,2例存活,1例失访。结论CDC是一种罕见的侵袭性肾细胞癌,预后极差。需要结合大体表现、组织形态及免疫表型与浸润性尿路上皮癌、SMARCB1缺陷型肾髓质癌、FH缺陷型肾细胞癌、乳头状肾细胞癌等鉴别。Objective To investigate the clinicopathological features,diagnosis,differential diagnosis and prognosis of collecting duct carcinoma of the kidney(CDC).Methods The clinicopathological features and immunohistochemical staining of 8 patients with CDC were analyzed retrospectively,and related literatures were further reviewed.Results There were 5 males and 3 females in this study,whose age ranged from 38 years to 78 years(mean 59 years).Gross hematuria,fever,anorexia and fatigue were the main clinical manifestations.Macroscopically,tumors were mainly 1ocated in the renal medulla and were solitary,with greyish-white and hard cut surfaces,measuring 3.8 cm to 6.3 cm in greatest diameter.Histopathologically,there were tubular,tubulopapillary,micropapillary,solid,and cord-like architectures with associated focal sarcomatoid areas,and desmoplatic sromal reaction.The desmoplastic stroma was present with intense inflammatory infiltrates,comprising lymphocytes,plasma cells,and neutrophils.Tumor cells were polygonal or hobnail,with marked cytological atypia and conspicuous nucleolus and numerous mitoses.Immunohistochemically,tumor cells were positive for CK19(8/8),CK7(8/8),34βE12(7/8),E-cadherin(7/8),vimentin(7/8)and retained expression of fumarate hydratase(FH)and SMARCB1(INI-1).During 3 months to 57 months of follow-up,4 patients died within 12 months,1 patient died of pulmonary fibrosis within 36 months after surgery,2 patients survived,and 1 patient lost.Conclusion CDC is a rare invasive renal cell carcinoma with poor prognosis.It is necessary to differentiate from invasive urothelial carcinoma,SMARCB1-deficient renal medullary carcinoma,FH-defificient renal cell carcinoma,and papillary renal cell carcinoma,in combination with macroscopic appearance,histopathology and immunophenotype.

关 键 词：肾集合管癌 临床病理 免疫组化 鉴别诊断 

分 类 号：R737.11[医药卫生—肿瘤]