儿童肾肿瘤多中心协作组288例诊治报告

目的:总结中国小儿肿瘤专业委员会(CCCG)肾母细胞瘤(WT)-2015方案的疗效。方法:前瞻性研究,在CCCG-WT-2009方案基础上修正建立WT-2015方案。总结2015年9月至2018年12月在14家协作组成员单位明确诊断的288例初发肾肿瘤患儿的临床资料。分析儿童肾肿瘤的发病年龄、病理亚型分布、分期、疗效及预后因素。生存曲线应用Kaplan-Meier法,单因素分析应用Log-Rank法。结果:288例肾肿瘤中WT 261例,其中良好组织学型(FH)WT 254例,占97.3%,间变型(UFH)WT 7例,占2.7%。FHWT、UFHWT 3年无事件生存率(EFS)分别为(88.9±2.1)%、(80.0±17.9)%,优于WT-2009方案的81.2%、71.7%。Ⅲ、Ⅳ期FHWT有放疗指征者96例,其中76例实施放疗,20例因未放疗升至M方案化疗(环磷酰胺、依托泊苷、更生霉素、长春新碱、阿霉素),放疗与未放疗患儿3年EFS分别为(84.7±4.3)%及(84.7±8.1)%,预后差异无统计学意义(χ2=0.015,P=0.902)。肾肉瘤包括肾透明细胞肉瘤22例和肾横纹肌样瘤5例。3年EFS分别为(94.4±5.4)%及(20.0±17.9)%。将年龄、性别、病理类型、分期、手术中破溃否、治疗结束是否达到完全缓解(CR)、放疗否进行预后单因素分析,结果发现病理类型(χ2=44.329,P<0.01)和治疗结束时是否达到CR(χ2=49.459,P<0.01)是独立预后影响因素。结论:CCCG-WT-2015方案与WT-2009方案相比,预后改善,可扩大协作组对象应用本方案。

Multicenter clinical study on the diagnosis and treatment of childhood renal tumor

Objective To summarize the effect of Chinese Children′s Cancer Group(CCCG)Wilms tumor(WT)-2015 protocol.Methods This was a prospective study.CCCG-WT-2015 protocol was revised on the basis of the CCCG-WT-2009 protocol.Clinical data of 288 children diagnosed with newly diagnosed kidney neoplasms in fourteen pediatric centers between September 2015 to December 2018 were summarized.The age of onset,distribution of pathological subtypes,staging,curative effect and prognostic factors of these children were analyzed.Kaplan-Meier method was used for survival curve and Log-Rank method was used for univariate analysis.Results Among 288 cases with kidney neoplasms,there were 261 cases of WT,including 254 cases(97.3%)with favorable histology(FH)WT and 7 cases(2.7%)with unfavorable histology WT(UFHWT).The 3 year events free survival(EFS)rate for FHWT and UFHWT were(88.9±2.1)%and(80.0±17.9)%,which were better than that in WT-2009(81.2%and 71.7%).In the 96 cases of stageⅢ/ⅣFHWT with indications for radiotherapy,76 cases received radiation,another 20 cases received M protocol chemotherapy(cyclophosphamide,etoposide,gentamycin,vincristine and adriamycin)instead of radiation.The 3 year EFS rate for these two groups were(84.7±4.3)%and(84.7±8.1)%(χ2=0.015,P=0.902).There were 22 renal clear cell sarcoma and 5 malignant rhabdoid tumor,3 year EFS rate of them was(94.4±5.4)%and(20.0±17.9)%.Univariate analysis was performed for age,gender,pathological type,stage,whether rupture occurred during operation,whether complete remission(CR)occurred at the end of treatment and radiotherapy.Pathological types(χ2=44.329,P<0.01)and failure to achieve CR at the end of the treatment(χ2=49.459,P<0.01)were independent factor for predicting survival.Conclusion Compared with CCCG-WT-2009,treatment of renal tumors in CCCG-WT-2015 study yielded good survival outcome,which can be further applied.