透明细胞乳头状肾细胞肿瘤临床病理研究  

Clinical and pathological study on clear cell papillary renal cell tumor

作　　者：彭森[1] 孙辉 王雪 王朝夫[2] 吴迪[1] PENG Sen;SUN Hui;WANG Xue;WANG Chaofu;WU Di(Department of Pathology,Xuzhou Central Hospital,Xuzhou Jiangsu 221000;Department of Pathology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]徐州市中心医院病理科,江苏徐州221000 [2]上海交通大学医学院附属瑞金医院病理科,上海200025

出　　处：《临床与病理杂志》2024年第2期261-267,共7页Journal of Clinical and Pathological Research

摘　　要：目的:透明细胞乳头状肾细胞肿瘤(clear cell papillary renal cell tumor,CCPRCT)是一种少见但重要的肾肿瘤类型,与其他肾细胞肿瘤具有类似的形态学特征,易导致误诊。本研究旨在探讨CCPRCT的临床病理特征、诊断及鉴别诊断要点,以提高其病理诊断的准确性。方法:收集15例CCPRCT患者,观察其临床及影像学特点,分析其镜下形态、免疫表型,并复习相关文献。结果:15例患者中,男10例,女5例,年龄为(54±17)岁,10例患者肿瘤位于左肾,5例患者肿瘤位于右肾。组织学上肿瘤均由增厚的纤维囊包裹,并局限于肾实质内;肿瘤细胞排列成乳头状、管状、囊状、腺泡及实性等结构;乳头由小到中等大小、透明细胞质的单层细胞组成,肿瘤均为世界卫生组织/国际泌尿病理学会(World Health Organization/International Society of Urological Pathology,WHO/ISUP)1级或2级;细胞核反极性分布,即核上移,靠近腔面,远离基底,类似鲨鱼牙齿排列。免疫组织化学染色显示:细胞角蛋白7、碳酸酐酶9(carbonic anhydrase 9,CA9)和高分子量细胞角蛋白(34βE12)均为阳性表达;α甲基酰基辅酶A消旋酶、CD117、转录因子E3均为阴性表达;CCPRCT中典型的CA9表现方式是“U型”着色,即腔缘不表达,基底和侧面表达。患者术后随访均未见复发或转移。结论:CCPRCT是一种惰性肾细胞肿瘤,预后好,临床可能存在过诊断,其形态学为特征性的核朝向腔缘的线性排列,特殊的免疫表型CA9呈“U型”阳性,可以与其他肾细胞肿瘤区分开来,但仍然需要积累更多患者来阐释其预后。Objective:Clear cell papillary renal cell tumor(CCPRCT)is a rare but significant type of renal tumor.It has similar morphological features to other renal cell tumors,which can easily lead to misdiagnosis.This study aims to investigate the clinical and pathological characteristics,diagnostic criteria,and differential diagnosis of CCPRCT to enhance the accuracy of its pathological diagnosis.Methods:A total of 15 cases of CCPRCT were collected,and their clinical and radiological features were observed.The microscopic morphology and immunophenotype were analyzed,and relevant literature was reviewed.Results:Among the 15 cases,10 were male and 5 were female,with age of(54±17)years.The tumors were located in the left kidney and five in the right.Histologically,all tumors were encapsulated by a thickened fibrous capsule and confined within the renal parenchyma.The tumor cells were arranged in papillary,tubular,cystic,acinar,and solid structures.The papillae ranged from small to medium-sized and were composed of a single layer of clear cells.All tumors were graded as World Health Organization/International Society of Urological Pathology(WHO/ISUP)grade 1 or 2.The cells exhibited reverse polarity,with nuclei positioned apically,similar to the arrangement of shark teeth.Immunohistochemical staining showed positive expression for cytokeratin 7,carbonic anhydrase 9(CA9),and high-molecular-weight cytokeratin(34βE12),negative expression forα-methylacyl-CoA racemase,CD117,and transcription factor E3.A typical U-shaped CA9 staining pattern was observed in CCPRCT,where the luminal edge was negative,but the base and lateral aspects were positive.No recurrences or metastases were observed during postoperative follow-up.Conclusion:CCPRCT is an indolent renal cell tumor with a favorable prognosis.There is a potential for overdiagnosis clinically.Its morphological hallmark is the characteristic linear arrangement of nuclei oriented towards the luminal edge,and its distinctive immunophenotype with U-shaped positive CA9 can differenti

关 键 词：透明细胞乳头状肾细胞肿瘤 病理学特征 诊断 鉴别诊断 

分 类 号：R737.11[医药卫生—肿瘤]