延胡索酸水合酶缺陷型肾细胞癌诊治的现状和展望  

Current status and perspective in the diagnosis and treatment of fumarate hydratase-deficient renal cell carcinoma

作　　者：王涛[1] 马鑫[1] 黄庆波[1] 丁效蕙[2] 董隽[1] 宋涛[1] 郭刚[1] 张旭[1] Wang Tao;Ma Xin;Huang Qingbo;Ding Xiaohui;Dong Juan;Song Tao;Guo Gang;Zhang Xu(Department of Urology,Chinese PLA General Hospital,Bejing 100039,China;Department of Pathology,First Medical Center,Chinese PLA General Hospital,Beijing 100039,China)

机构地区：[1]解放军总医院泌尿外科医学部,北京100039 [2]解放军总医院第一医学中心病理科,北京100039

出　　处：《中华泌尿外科杂志》2023年第6期466-470,共5页Chinese Journal of Urology

基　　金：国家自然科学基金(81972389)。

摘　　要：延胡索酸水合酶缺陷型肾细胞癌(FH-RCC)是一种罕见且具有高侵袭性和转移性的肾细胞癌,临床诊断相对困难,对于晚期FH-RCC缺乏标准的系统治疗方案。因此,在临床工作中,需要更深入地了解该病,充分结合家族史、影像学、病理学表现进行临床识别,必要时进行分子病理检测通过根治性手术或系统治疗方案,可以改善患者的预后。Fumarate hydratasedeficient renal cell carcinoma(FH-RCC)is s a rare and highly aggressive and metastatic form of renal cell carcinoma.Clinical diagnosis is relatively challenging,and there is a lack of recognized systemic treatment options.Therefore,in clinical practice,it is necessary to have a deeper understanding of this disease and fully integrate family history,imaging findings,and pathological manifestations for clinical identification,and genetic testing shoule be conducted when necessary.Improved patient prognosis can be achieved through the selection of appropriate curative surgery or systemic treatment strategies.

关 键 词：癌 肾细胞 交延胡索酸水合酶缺陷型肾细胞癌 讠诊断 治疗 预后 

分 类 号：R737.11[医药卫生—肿瘤]