6例成人后肾腺瘤的临床病理特征  

Clinical and pathological characteristics of 6 cases of adult metanephric adenoma

作　　者：陈倩倩 郑瑾玥 罗雯雯 CHEN Qianqian;ZHENG Jinyue;LUO Wenwen(Department of Pathology,Third Affiliated Hospital,Sun Yat-sen University,Guangzhou 510000,China)

机构地区：[1]中山大学附属第三医院病理科,广州510000

出　　处：《临床与病理杂志》2025年第1期104-109,共6页Journal of Clinical and Pathological Research

摘　　要：目的:成人后肾腺瘤是一种罕见的肾脏肿瘤,预后良好,但容易误诊。本研究探讨成人后肾腺瘤的临床病理特征、免疫表型、分子病理特点、鉴别诊断及预后,旨在提高病理医师对该肿瘤的认识。方法:收集中山大学附属第三医院6例成人后肾腺瘤的临床资料,对其标本行HE染色、免疫组织化学染色和分子检测,并复习相关文献。结果:女性4例,男性2例,男女比为1꞉2,年龄47.17(26~66)岁。5例行部分肾切除,1例全肾切除。镜下检查示:肿瘤由体积较小的圆形或卵圆形细胞构成,呈小管状或乳头状排列,细胞形态较一致,胞质稀少,核分裂象罕见。5例边界清楚;1例与正常肾分界不清,外院误诊为成人上皮型肾母细胞瘤。免疫组织化学检查示:6例均表达细胞角蛋白(cytokeratin,CK)CAM5.2、肾母细胞瘤基因1(Wilms tumor 1,WT1)、鼠类肉瘤滤过性毒菌致癌基因同源体B1(v-Raf murine sarcoma viral oncogene homolog B1,BRAFV600E)、波形蛋白(vimentin);5例表达髓鞘相关糖蛋白57(CD57),神经细胞黏附分子(neural cell adhesion molecule,CD56)、细胞角蛋白7(cytokeratin 7,CK7)及α-甲基酰基-辅酶A消旋酶(α-methylacyl-CoA racemase,P504S)局灶或阴性表达;均不表达上皮细胞膜抗原(epithelial membrane antigen,EMA)、结蛋白(desmin)、酪氨酸激酶生长因子受体蛋白(CD117)、共同的急性淋巴母细胞白血病抗原(common acute lymphoctte leukemia antigen,CD10)、碳酸酐酶9(carbonic anhydase IX,CAIX),细胞增殖相关蛋白Ki-67呈低表达,Ki-67阳性率为1%~5%。二代测序(next-generation sequenching,NGS)或反转录聚合酶链反应(reverse transcription polymerase chain reaction,RT-PCR)检测到6例均有BRAFV600E突变。随访7~96个月,均未复发或转移。结论:后肾腺瘤是一种良性肿瘤,预后好,未见复发或转移,易误诊为上皮型肾母细胞瘤。Objective:Adult metanephric adenoma is a rare renal tumor with a good prognosis,but which is easily misdiagnosed.This study aims to explore the clinical pathology,immunophenotype,molecular pathological characteristics,differential diagnosis,and prognosis of adult metanephric adenoma,with the goal of increasing pathologists’awareness of this tumor.Methods:The clinical data of 6 cases of adult metanephric adenoma from the Third Affiliated Hospital of Sun Yat-sen University were collected.HE staining,immunohistochemistry,and molecular testing were performed on the specimens,and relevant literature was reviewed.Results:Among the 6 cases,4 were female and 2 were male,with a male-to-female ratio of 1꞉2.The mean age was 47.17(range 26−66)years.Five cases underwent partial nephrectomy,and one case underwent total nephrectomy.Microscopic examination showed that the tumor was composed of small,round or oval cells arranged in tubular or papillary structures.The cell morphology was relatively uniform,with sparse cytoplasm,and mitotic figures were rare.The boundaries were clear in 5 cases,while one case had an unclear boundary with the normal kidney and was misdiagnosed as adult epithelial-type nephroblastoma at an outside hospital.Immunohistochemistry showed that all 6 cases expressed cytokeratin(CK)CAM 5.2,Wilms tumor 1(WT 1),v-Raf murine sarcoma viral oncogene homolog B 1(BRAF V 600 E),and vimentin.Five cases expressed CD 57,neural cell adhesion molecule(CD 56),cytokeratin 7(CK 7)andα-methylacyl-CoA racemase(P 504 S)focally or negatively,while none expressed epithelial membrane antigen(EMA),desmin,CD 117,common acute lymphoctte leukemia antigen(CD 10),or carbonic anhydase IX(CAIX).The Ki-67 proliferation index was low,with a positive rate of 1%−5%.Next generation sequencing(NGS)or reverse transcription PCR(RT-PCR)detected BRAF V 600 E mutations in all 6 cases.Follow-up lasted for 7−96 months,and no recurrence or metastasis was observed.Conclusion:Metanephric adenoma is a benign tumor with a good prognosis and no

关 键 词：后肾腺瘤 临床病理特征 免疫组织化学 鼠类肉瘤滤过性毒菌致癌基因同源体B1 上皮型肾母细胞瘤 

分 类 号：R737.11[医药卫生—肿瘤]