单中心连续79例儿童单侧组织学良好型肾母细胞瘤先期手术治疗结果及疗效分析

Surgical and oncological outcome of 79 consecutive patients with unilateral favorable histology Wilms tumor treated in one single institution following an upfront tumor surgical strategy

目的·加深对先期手术策略治疗儿童肾母细胞瘤(Wilms tumor,WT)的理解,规范手术操作及其后的化学治疗±放射治疗(放疗)流程,为中国儿童WT临床研究提供必要的数据支持。方法·回顾性收集2010年1月1日—2017年12月30日首诊于上海交通大学医学院附属上海儿童医学中心肿瘤外科并获得病理诊断的初治单侧组织学良好型WT(favorable histology Wilms tumor,FHWT)连续病例资料。所有病例均按照中国小儿肿瘤专业委员会(Chinese Children Cancer Group,CCCG)-WT-2009和CCCG-WT-2015方案诊断、分期并接受相应治疗。采用SPSS 25.0软件对手术结果、无事件生存率(event-free survival,EFS)和总体生存率(overall survival,OS)进行分析。最后随访时间为2020年3月31日。结果·共纳入79例初治单侧FHWT连续病例。男性41例,女性38例;中位年龄27.1个月。系统分期Ⅰ期7例,Ⅱ期39例,Ⅲ期23例,Ⅳ期10例。69例(87.3%)一期手术行肾脏肿瘤切除术,10例(12.7%)行肾脏肿瘤活检术(2例粗针穿刺活检,8例开腹活检)。11例患儿出现疾病进展/复发,1例放弃治疗后8周又回到原治疗方案。具有原发灶放疗指征的32例患儿中27例实际接受放疗,10例初诊时有肺转移灶患儿中3例接受全肺放疗。67例无事件病例中位随访时间为69.8个月。所有病例5年EFS和OS分别为84.8%和92.4%;Ⅰ、Ⅱ、Ⅲ以及Ⅳ期患儿5年EFS分别为100.0%、84.6%、95.7%和50.0%(P=0.010),5年OS分别为100.0%、94.9%、95.7%和70.0%(P=0.070)。Ⅲ/Ⅳ期患儿中,一期手术切除原发灶病例5年EFS高于仅实施活检术患儿(91.3%vs 60.0%,P=0.032);对于先期接受活检术患儿,6周评估可进行根治性手术者较无法根治性手术者5年EFS更高(100%vs 0,P=0.005)。结论·采用先期手术策略治疗儿童单侧FHWT安全、可行且有效。一期手术切除肾原发病灶及准确的淋巴结取样是确定分期和成功治疗的关键。

Objective·To improve the understanding of upfront surgical strategy in the treatment of Wilms tumor(WT)and standardize the upfront resection procedure and subsequent chemotherapy and/or radiotherapy approach,providing necessary data support for clinical study in childhood WT in China.Method·From January 1,2010 to December 30,2017,the clinical records of patients with unilateral favorable histology WT(FHWT)who were initially diagnosed at Shanghai Children's Medical Center,Shanghai Jiao Tong University School of Medicine were retrospectively collected.All cases were diagnosed,staged and treated according to the Chinese Children Cancer Group(CCCG)-WT-2009 or CCCG-WT-2015 Protocol.Surgical outcome,event-free survival(EFS)and overall survival(OS)were analyzed using SPSS software,version 25.0.Follow-up was updated to March 31,2020.Result·A total of 79 consecutive cases with unilateral FHWT were enrolled,including 41 males and 38 females,with a median age of 27.1 months.Systemic staging showed that there were 7 cases with stageⅠ,39 cases with stageⅡ,23 cases with stageⅢand 10 cases with stageⅣ.Sixty-nine patients(87.3%)underwent nephrectomy and 10 patients(12.7%)underwent biopsy of renal tumor tissue(2 with core needle biopsy and 8 with open biopsy)in the primary operation.Eleven cases experienced disease progression/recurrence and 1 abandoned treatment and returned to original treatment plan after 8 weeks.Local radiation therapy was applied to 27 of 32 patients with indication.Only 3 of 10 patients with lung metastasis at diagnosis received whole lung radiation therapy.A median follow-up of 69 cases without incident was 69.8 months.The 5-year EFS and OS of all cases were 84.8%and 92.4%respectively.The 5-year EFS of children with stageⅠ,Ⅱ,ⅢandⅣwere 100.0%,84.6%,95.7%and 50.0%(P=0.010),respectively.And the 5-year OS of children with stageⅠ,Ⅱ,ⅢandⅣwere 100.0%,94.9%,95.7 and 70.0%(P=0.070),respectively.The 5-year EFS of children with stageⅢ/Ⅳreceiving initial nephrectomy was higher than that of those undergoing biopsy(91.3%vs 60.0%,P=0.032).The 5-year EFS of children with initial biopsy,who underwent nephrectomy at week 6 was significantly higher than that of those who did not(100%vs 0,P=0.005).Conclusion·The upfront tumor surgical strategy of unilateral WT is safe,feasible and effective in the treatment of FHWT.Initial nephrectomy and clearance of all local diseases with lymph nodal sampling is the key to staging and successful treatment.