儿童双侧肾母细胞瘤诊治分析  

Diagnosis and treatment of bilateral Wilms'tumor

作　　者：李振武[1] 张潍平[1] 屈彦超[1] 汪添益 宋宏程[1] 孙宁[1] 田军[1] 李明磊[1] Li Zhenwu;Zhang Weiping;Qu Yanchao;Wang Tianyi;Song Hongcheng;Sun Ning;Tian Jun;Li Minglei(Department of Urology,Affiliated Beijing Children's Hospital,Capital Medical University,National Children's Medical Center,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院泌尿外科,北京100045

出　　处：《中华小儿外科杂志》2022年第7期630-634,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨儿童双侧肾母细胞瘤(bilateral wilms tumor, BWT)的临床特点、治疗效果以及预后, 加深临床认知。方法收集北京儿童医院在2008年1月至2019年12月收治的43例BWT患儿的临床资料, 包括年龄、临床表现、影像学检查、术前化疗、手术方式、病理诊断和预后。43例共86侧肾脏;男24例, 女19例;就诊中位年龄为17个月, 范围为3~69个月;术前化疗30例(60侧)作为术前化疗组, 未化疗9例(18侧)作为未化疗组, 余4例(8侧)资料不详不列入任何组别;6例化疗前行穿刺活检。30例(60侧)术前化疗患儿中术前化疗反应评估资料完整26例(52侧), 缺失4例(8侧)。术前化疗时间范围为4~12周, 术前化疗方案以VA方案(长春新碱+更生霉素)为主, 共19例(38侧);VAD方案(长春新碱+更生霉素+阿霉素), 9例(18侧);仅运用长春新碱化疗, 2例(4侧)。本研究病例随访数据采用Kaplan-Meier法绘制生存曲线, SPSS 24.0软件计算4年无事件生存率和总体生存率。结果行双侧保留肾单位手术(nephron sparing surgery, NSS) 30例[69.8%(30/43)];行一侧瘤肾切除术+对侧NSS有13例[30.2%(13/43)]。术前化疗组中双侧NSS占80.0%(24/30), 术前未化疗组中双侧NSS有6例。43例患儿术后均复查增强CT, 双侧肾脏残存肾实质累计大于单侧肾脏23例[53.5%(23/43)], 双侧肾脏残存肾实质累计小于单侧肾脏20例[46.5%(20/43)]。所有患儿均获随访, 中位随访时间为48个月, 范围为8~158个月;中位随访年龄为71个月, 范围为39~180个月。存活39例, 死亡4例;存活患儿中, 肾功能衰竭1例, 带瘤存活4例;死亡患儿中, 1例为术后9年无瘤状态下死于脑疝, 余3例为复发治疗后死亡。术后复发9例, 单侧最高分期为Ⅲ期4例, Ⅳ期5例;复发部位为原位复发7例, 腰大肌处复发1例, 腹膜后多处复发1例;中位复发时间为11个月, 范围为1~38个月;7例手术切除复发灶, 术后加用依托泊苷、卡铂、环磷酰胺加强化疗;复�Objective To explore the clinical characteristics,therapeutic outcomes and prognoses of bilateral Wilms tumor(BWT)in children and to enhance its clinical awareness.Methods From January 2008 to December 2019,clinical data were retrospectively reviewed for 43 hospitalized BWT children,including age,clinical manifestations,imaging findings,preoperative chemotherapy,surgical approaches,pathological diagnoses and prognoses.With a total of 86 kidneys,there were 24 boys and 19 girls with an onset age of 17(3-69)months.Thirty children(60 sides)on preoperative chemotherapy were selected as preoperative chemotherapy group,9 cases(18 sides)not on chemotherapy as non-chemotherapy group and the remaining 4(8 sides)with unknown data not grouped.Six children underwent needle biopsy before chemotherapy.Among 30 children(60 sides)with preoperative chemotherapy,26(52 sides)had complete assessment data of preoperative chemotherapy response and 4(8 sides)were missing.The duration of preoperative chemotherapy was 4 to 12 weeks.The major preoperative chemotherapy regimens were VA(vincristine+dactinomycin)(n=19,38 sides);VAD(vincristine+dactinomycin+adriamycin)(n=9,18 sides)and vincristine(n=2,4 sides).Survival curves of follow-up data were plotted with the Kaplan-Meier method and 4-year event-free and overall survival rates calculated by SPSS 24.0 software.Results Nephron sparing surgery(NSS,n=30)and unilateral tumor nephrectomy plus NSS(n=13)were performed.Preoperative chemotherapy accounted for 80.0%(24/30)of NSS and there were 6 cases of NSS without chemotherapy.Cumulative residual renal parenchyma of kidney was greater than that of unilateral kidney[53.5%(23/43)]while cumulative residual renal parenchyma of kidney was less than that of unilateral kidney[46.5%(20/43)].The follow-up period was 48(8-158)months.And the follow-up age was 71(39-180)months.Except for 4 deaths,the remainders survived.Among survivors,there were renal failure(n=1)and surviving with tumor(n=4).Among 9 cases of postoperative recurrence,there were death(n=3),s

关 键 词：WILMS瘤 儿童 化学治疗 

分 类 号：R737.11[医药卫生—肿瘤]