延胡索酸水合酶缺陷型肾细胞癌伴瘤栓的诊治经验和预后分析  

Diagnosis,treatment and prognosis of FH-deficient renal cell carcinoma with tumor thrombus

作　　者：邱敏[1] 田晓军[1] 贺慧颖[2] 赵梅莘[3] 王滨帅[1] 董靖晗 刘承[1] 马潞林[1] 张树栋[1] Qiu Min;Tian Xiaojun;He Huiying;Zhao Meixin;Wang Binshuai;Dong Jinghan;Liu Cheng;Ma Lulin;Zhang Shudong(Department of Urology,Peking University Third Hospital,Bejing 100191,China;Department of Pathology,Peking University Third Hospital,Beijing 100191,China;Department of Nuclear Medicine,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院泌尿外科,北京100191 [2]北京大学第三医院病理科,北京100191 [3]北京大学第三医院核医学科,北京100191

出　　处：《中华泌尿外科杂志》2023年第6期416-421,共6页Chinese Journal of Urology

摘　　要：目的探讨延胡索酸水合酶缺陷型肾细胞癌(FH-deficient RCC)伴瘤栓的诊治经验和预后。方法回顾性分析2019年8月至2022年10月北京大学第三医院收治的6例FH-deficient RCC伴瘤栓患者的病例资料,男4例,女2例。年龄22~57岁,其中<40岁2例。肿瘤位于左侧5例,右侧1例。肿瘤最大径8(4.8,14.0)cm。完善增强CT等相关检查后行手术治疗。本组6例中,1例行开放手术,术中发现下腔静脉粘连重,仅姑息切除左肾;余5例中,1例行后腹腔镜根治性右肾切除术+瘤栓切除术,1例行经腹腔途径腹腔镜根治性左肾切除术+瘤栓切除术,3例行机器人辅助腹腔镜根治性左肾切除术+瘤栓切除术。结果手术时间293(185,366)min。2例术中需要阻断腔静脉,阻断时间分别为13min和28min。肾肿瘤和瘤栓病理诊断为FH-deficient RCC。病理检查:大体标本切面为灰白、灰黄色,实性,常伴坏死,局灶可见囊腔;镜下观察见肿瘤广泛累及肾实质,肿瘤呈乳头状、筛状和管囊状结构。免疫组化染色检查FH(-)2SC(+)。术后住院时间8(4,15)d。中位随访时间13(4,27)个月。1例姑息切除左肾者术后行靶向药物治疗+放疗,术后15个月因消化道穿孔死亡。余5例中,4例出现转移,采用系统治疗,其中1例术后27个月死亡;1例未发现转移,随访中发现子宫肌瘤。结论FH-deficient RCC伴瘤栓者罕见,本病侵袭性较强,术前诊断困难,临床预后差。手术联合系统治疗是治疗FH-deficient RCC伴瘤栓的有效方式。Objective To Explore the diagnosis,treatment and prognosis of FH-deficient renal cell carcinoma(FH-deficient RCC)with tumor thrombus,and share surgical experience.Methods From August 2019 to October 2022,6 cases of FH-deficient RCC with tumor thrombus were diagnosed and treated in our center,including 4 males and 2 females.The patients were aged 22 to 57 years,with 2 cases younger than 40 years,icluding 5 cases on the left and 1 case on the right.The median maximum diameter of the tumor is 8(4.8,14.0)cm.Operations were performed after complete examination(enhanced CT and other related examinations).One case underwent open surgery and palliative resection of the left kidney was performed because of severe adhesion of the inferior vena cava.Among the remaining 5 cases,1 case underwent retroperitoneal laparoscopic right radical nephrectomy with inferior vena cava thrombectomy,1 case underwent transabdominal laparoscopic left radical nephrectomy with inferior vena cava thrombectomy,and 3 cases underwent robot assisted laparoscopic left radical nephrectomy with inferior vena cava thrombectomy.Results The median surgical time was 293(185,366)min,with blockage of the vena cava for 13 min and 28 min in 2 of 6 cases,respectively.The pathological report of renal tumor and tumor thrombus was FHdeficient renal carcinoma.The pathological features were as follows:the gross section of the specimen was gray yellow solid,often accompanied by necrosis,and the cystic cavity could be seen locally.Microscopically,the tumor extensively involved the renal parenchyma,with papillary,cribriform and tubular cystic structures.Immunohistochemistry showed FH(-),2SC(+).The median postoperative hospital stay was 8(4,15)days.The median follow-up time was 13(4,27)months.One patient undergoing palliative resection of the left kidney underwent targeted therapy and radiotherapy after surgery(died 15 months after surgery due to gastrointestinal perforation).During the follow-up process,4 cases experienced metastasis and received systematic treatment,

关 键 词：癌 肾细胞 延胡索酸水合酶缺陷型肾癌 瘤栓 治疗 预后 

分 类 号：R737.11[医药卫生—肿瘤]