以广泛多房囊性结构为特征的肾肿瘤的临床病理观察  

Renal tumors characterized by extensive multilocular cystic structures:a clinicopathological study

作　　者：王露[1] 李惠[1] 章宜芬[1] WANG Lu;LI Hui;ZHANG Yi-fen(Department of Pathology,Jiangsu Province Hospital of Chinese Medicine,Nanjing 210004,China)

机构地区：[1]江苏省中医院病理科,南京210004

出　　处：《诊断病理学杂志》2023年第6期560-563,576,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨以广泛多房囊性结构为特征的肾肿瘤的临床病理特征。方法回顾性分析本院以广泛多房囊性结构为特征的肾肿瘤,包括:1例MED15-TFE3融合基因的Xp11易位肾细胞癌(RCC)、1例管状囊性肾细胞癌(TCRCC)及6例低度恶性潜能的多房囊性肾肿瘤(MCRN-LMP)的临床资料、病理检查及分子检测结果。结果入组的3种肾肿瘤大体均为境界清楚的广泛多房囊性结构。其中MED15-TFE3融合基因的RCC镜下可见胞质透明到嗜酸性的低级别肿瘤细胞呈乳头状、腺泡状或者巢状分布于纤维囊壁;砂砾体易见;免疫组化特征性显示TFE3强阳性表达;高通量测序结果显示MED15-TFE3基因融合。TCRCC巨检有特征性的海绵状外观,镜下可见肿瘤由厚薄不一的纤维囊壁分隔成大小不等的管囊结构,囊腔壁可见扁平至靴钉样的嗜酸性肿瘤细胞;免疫组化染色Cathepsin K、CD10、P504s、Pax-8、Vimentin阳性表达,TFE3和TFEB均为阴性表达。MCRN-LMP镜下观察肿瘤也是由纤细的纤维间隔分隔,囊壁被覆单层透明低级别肿瘤细胞,纤维间隔内无膨胀性生长肿瘤细胞结节;免疫组化显示肿瘤细胞PAX8和CA9强阳性表达,TFE3和TFEB均为阴性表达。结论MED15-TFE3融合基因的Xp11易位RCC、TCRCC和MCRN-LMP大体上均是广泛多房囊状结构,组织学形态也部分相似,但是免疫组化及分子结果有所不同,有助于鉴别诊断。Objective To investigate the clinicopathological features of renal tumors characterized by extensive multilocular cystic structures.Methods The clinical data,pathological examination and molecular detection results of renal tumors characterized by extensive multilocular cystic structures in our hospital were analyzed retrospectively,including one case of XP11 translocation renal cell carcinoma(RCC)with MED15-TFE3 fusion gene,one case of tubular cystic renal cell carcinoma(TCRCC)and six cases of multilocular cystic renal tumor of low malignant potential(MCRN-LMP).Results The three types of renal tumors included in the study were all broad multilocular cystic structures with clear boundary.Among them,RCC with MED15-TFE3 fusion gene showed that the low-grade tumor cells with clear to eosinophilic cytoplasm were papillary,acinar or nesting in the wall of the fibrous capsule under microscope.Psammoma bodies were easily noted.The strong expression of TFE3 was characterized by immunohistochemistry.High throughput sequencing showed MED15-TFE3 gene fusion.The TCRCC macro examination showed a characteristic spongy appearance.Microscopically,the tumor was divided into a tubular structure of different sizes by the fibrous capsule wall of different thickness.The cystic wall showed eosinophilic tumor cells that were flat to boot nail like.Immunohistochemical staining showed positive expression of cathepsin K,CD10,P504s,Pax-8,Vimentin,and negative expression of TFE3 and TFEB.Microscopic observation of MCRN-LMP showed that the tumor was also separated by thin fibrous septum,and the cystic wall was covered with a single layer of low-grade clear tumor cells.There was no tumor cell nodule in an expansive growth pattern in the fibrous septum.Immunohistochemistry showed that PAX8 and CA9 were strongly positive,while TFE3 and TFEB were negative.Conclusion The Xp11 translocation of MED15-TFE3 fusion gene in renal cell carcinoma,TCRCC and MCRN-LMP generally presents extensive multilocular cystic structures,and the histological morpholog

关 键 词：MED15-TFE3融合基因的Xp11易位肾细胞癌 管状囊性肾细胞癌 低度恶性潜能的多房囊性肾肿瘤 高通量测序 

分 类 号：R737.11[医药卫生—肿瘤]