肾集合管癌十例临床病理分析

Clinicopathologic features with collecting duct carcinoma of kidney： report of 10 cases

目的探讨肾集合管癌的病理学特征、免疫表型、鉴别诊断及预后。方法回顾性分析南京医科大学第一附属医院2009年1月至2017年8月收集的10例肾集合管癌患者的临床影像学、病理形态学、免疫组织化学表达及随访情况，并复习相关文献。结果肾集合管癌的临床症状无特殊性．影像学常为占位性表现，确诊依赖于病理学检查。肿瘤主要位于肾髓质，切面灰白质硬，常伴出血坏死。镜下示小管状或小管乳头状结构，可伴有肉瘤样区域，间质伴促结缔组织增生反应及淋巴浆细胞浸润；肿瘤细胞异型性显著，核级别高，核仁明显，核分裂象多见。免疫组织化学高表达细胞角蛋白（CK）19、E-cadherin、波形蛋白、高相对分子质量细胞角蛋白（HCK）、CK7、PAX8。治疗以根治性。肾切除手术为主．随访7例患者死亡，中位生存期10个月。结论。肾集合管癌是临床少见的高度侵袭性的肾恶性肿瘤，预后不良，以病理形态学及免疫组织化学为金标准做排除性诊断，并需与多种肾脏肿瘤等鉴别。

Objective To study the pathological features, immunophenotypes, differential diagnoses and prognostic parameters of collecting duct carcinoma of the kidney （CDC）. Methods Clinical imaging, histopathology, immunohistochemistry, and survival data of 10 patients at First Affiliated Hospital of Nanjing Medical University from January 2009 to August 2017 were retrospectively analyzed along with a review of literatures. Results The clinical symptoms of CDC were not specific, and image examinations showed space-occupying mass lesions. Tumors were mainly located in renal medulla with grey and firm cut face and the presence of focal hemorrhage and necrosis. Microscopically, there were predominant tubular or tubular-papillary structures with associated focal sarcomatoid areas, desmoplastic stromal reaction and lymphoplasmaeytic cells infiltration. Tumor cells had marked cytological atypia with high grade nuclei, conspicuous nucleolus and numerous mitoses. Immunohistochemieally, tumor cells were strongly positive for CK19, E-cadherin, vimentin, HCK, CK7 and PAXS. The main treatment was radical nephrectomy in the patients. Seven cases died of CDC with median survival of 10 months. Conclusions CDC is a rare, highly aggressive malignancy of kidney with poor prognosis. Definitive diagnosis should be made by histology and immunohistochemistry. Differential diagnoses include papillary renal cell carcinoma （type Ⅱ ）, renal medullary carcinoma, infiltrating high grade urothelial carcinoma, renal pelvis adenoearcinoma and metastatic adenocarcinomas.