目的 总结WT- 2016方案治疗Wilms肿瘤的疗效。方法 回顾分析2017年3月至2019年3月，采用CCCGWT- 2016 方案治疗的 Wilms 肿瘤患儿的临床资料，患儿随访至 2020 年4月1日，以Kaplan-Meier 曲线描述生存结局。 结果　共纳入43例患儿，男23例、女20例，左侧发病23例、右侧发病19例、双侧发病1例，中位年龄2.40（0.83~2.00）岁。 I期7例、II期7例、III期21例、IV期6例、V期2例；病理分型FH型38例、uFH型5例。随访中位时间24.13（17.23 ~31.10）月， 除1例复发患儿需继续强化治疗外，其余患儿已结束治疗。死亡4例（9.3%），2例死于复发、1例死于疾病进展、1例死于 脓毒症休克；复发4例（9.3%），3例转移至肺部（其中1例肿瘤原位复发合并肺部转移）、1例转移至纵膈。估计2年总生存 率为（93.02±3.89）%，2年无复发生存率为（90.58±4.49）%。总体脓毒症发生率14.0%，呼吸道感染发生率18.6%，药 物性肝损害发生率7.0%，4级骨髓抑制发生率39 . 5 %。Ⅳ级骨髓抑制各种不良反应的发生率分别为白细胞降低32 . 6 %， 中性粒细胞绝对值降低34 .9%，血小板降低23 .3%，血红蛋白降低9.3%。结论　采用WT- 2016方案治疗儿童Wilms肿瘤 预后较好。

Objective? To summarize the efficacy of WT- 2016 regimen in the treatment of Wilms tumor. Methods The clinical data of Wilms tumors treated with CCCG-WT- 2016 regimen from March 2017 to April 2019 were retrospectively analyzed. The children were followed up until April 1 , 2020 , and survival outcomes were described by Kaplan-Meier method. Results A total of 43 children ( 23 boys and 20 girls) were included, with a median age of 2 . 40 ( 0 . 83 ~ 2 . 00 ) years. The left, right and bilateral sides were affected in 23 , 19 and 1 patients respectively. There were 7 cases of stage Ⅰ, 7 cases of stage Ⅱ, 21 cases of stage Ⅲ, 6 cases of stage Ⅳ and 2 cases of stage Ⅴ. The pathological classification results were 38 cases of favorable histology (FH) type and 5 cases of unfavorable histology (uFH) type. The median follow-up of all patients was 24 . 13 ( 17 . 23~ 31 . 10 ) months. Except 1 relapsed child who needed further intensive treatment, the rest of the children had completed the treatment. There were 4 deaths ( 9 . 3 %), of which 2 were due to recurrence, 1 due to disease progression and 1 due to septic shock. There were 4 cases of recurrence ( 9 . 3 %), including 3 cases of metastasis to the lungs ( 1 case of in-situ tumor recurrence with pulmonary metastasis) and 1 case of metastasis to the mediastinum. Estimated 2 -year overall survival (OS) and progressionfree survival (PFS) was ( 93 . 02 ± 3 . 89 ) % and ( 90 . 58 ± 4 . 49 ) % respectively for all patients. The overall incidence of sepsis, respiratory infection, drug-induced liver injury and grade 4 myelosuppression were 14 . 0 %, 18 . 6 %, 7 . 0 %, and 39 . 5 % consecutively. The adverse reactions of grade 4 myelosuppression were the decreases in leukocytes by 32 . 6 %, neutrophils absolute value by 34 . 9 %,, platelets by 23 . 3 % and hemoglobin by 9 . 3 %. Conclusion WT- 2016 regimen has improved the prognosis of children with Wilms tumor.