肉瘤样肾细胞癌的致病机制与治疗进展  

Pathogenesis and therapeutic progress in sarcomatoid renal cell carcinoma

作　　者：陈旭(综述) 贺慧颖(审校) Xu Chen;Huiying He(Department of Pathology,School of Basic Medical Sciences,Peking University Health Science Center,Beijing 100191,China;Department of Pathololy,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学医学部病理学系,北京市100191 [2]北京大学第三医院病理科

出　　处：《中国肿瘤临床》2023年第9期483-486,共4页Chinese Journal of Clinical Oncology

摘　　要：肉瘤样肾细胞癌是一类伴有肉瘤样特征的肾癌,具有强侵袭性与不良预后,其发生与上皮间质转化有关。可能涉及的突变包括TP53、NF2、BAP1、ARID1A等,表观遗传调控异常可能也是触发肉瘤样转化的原因。肉瘤样肾细胞癌的治疗手段包括手术、靶向治疗、免疫治疗等,由于其具有高肿瘤突变负荷,并且PD-1与PD-L1的表达明显较高,针对PD-1/PD-L1的免疫检查点抑制剂(immune checkpoint inhibitors,ICIs)是重要的治疗手段。本文将对肉瘤样肾细胞癌的发生机制与PD-1/PD-L1的ICIs治疗机制及进展进行综述。Sarcomatoid renal cell carcinoma(sRCC)is an uncommon kind of renal cell carcinoma with sarcomatoid differentiation,which confers aggressiveness and a dismal prognosis.The epithelial–to-mesenchymal transition(EMT)is related to the sarcomatoid change of renal cell carcinoma.Driver mutations are noted in TP53,NF2,BAP1,and ARID1A.Abnormal epigenetic regulations may also trigger sarcomatoid transformation.Treatment includes surgery,targeted therapy,and immunotherapy.With a higher tumor mutational burden and a high expression of PD-1/PD-L1,PD-1 or PD-L1,immune checkpoint inhibitors(ICIs)-based regimens play an important role in the treatment of sRCC.In this review,we summarize the pathogenesis of sRCC and the mechanism of PD-1 or PD-L1 ICIsbased immunotherapy.

关 键 词：肉瘤样肾细胞癌 致病机制 免疫治疗 

分 类 号：R737.11[医药卫生—肿瘤]