AbstractInvestigators from the International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) report on outcomes of children with bilateral Wilms tumour treated on the SIOP 2001 study. They demonstrate that vincristine and actinomycin-D induction chemotherapy is sufficient in a subset of children, but most required additional agents during their treatment..

摘要国际儿科肿瘤学会肾肿瘤研究小组（SIOP-RTSG）的研究人员报告了SIOP 2001研究中治疗的双侧肾母细胞瘤儿童的结果。他们证明长春新碱和放线菌素D诱导化疗在一部分儿童中是足够的，但大多数在治疗过程中需要额外的药物。。

In both Europe and North America, neoadjuvant chemotherapy and bilateral nephron-sparing surgery are employed in the treatment of bilateral Wilms tumour (BWT) to preserve the greatest possible volume of functioning renal parenchyma [1]. Children with BWT are prone to develop diffuse anaplasia or to experience late disease relapse (>2 years) at higher rates than children with unilateral Wilms tumour [2].

在欧洲和北美，新辅助化疗和双侧肾单位保留手术被用于治疗双侧肾母细胞瘤（BWT），以保留尽可能大的功能性肾实质体积（1）。BWT患儿比单侧肾母细胞瘤患儿更容易发生弥漫性不典型增生或晚期疾病复发（>2年）。

These late relapses may be metachronous, “new” tumours due to germline or mosaic Wilms tumour genetic or epigenetic predisposition in patients with BWT [3, 4]. Furthermore, children with BWT experience higher rates of late renal failure resulting in the need for dialysis or kidney transplant when compared to patients with unilateral Wilms tumour [5].

由于BWT患者的种系或镶嵌Wilms肿瘤遗传或表观遗传易感性，这些晚期复发可能是异时的“新”肿瘤[3，4]。此外，与单侧肾母细胞瘤患者相比，BWT儿童晚期肾衰竭的发生率更高，因此需要透析或肾移植。

Nevertheless, the imperative to preserve kidney function must be balanced against oncologically sound treatment aimed to eradicate tumour and optimise event-free and overall survival. Studies conducted primarily in Europe through the International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) and in North America through the Children’s Oncology Group (COG) have aimed to determine the optimal therapeutic approach, sequence, and intensity for patients with BWT.In 2017, results were published from the COG Study AREN0534, which was the first prospective, multi-institutional clinical trial for children with BWT [6].

然而，保护肾功能的必要性必须与旨在根除肿瘤并优化无事件和总体生存的肿瘤学合理治疗相平衡。主要通过国际儿科肿瘤学会肾肿瘤研究小组（SIOP-RTSG）在欧洲和通过儿童肿瘤小组（COG）在北美进行的研究旨在确定BWT患者的最佳治疗方法，顺序和强度。2017年，COG研究AREN0534公布了结果，这是第一个针对BWT儿童的前瞻性多机构临床试验（6）。

This study aimed to improve event-free and overall survival while maximising renal preservation in children with BWT by intensifying preoperative chemotherapy to include vincristine, actinomycin-D and doxorubicin (VAD), incorporating information from post-chemotherapy treatment histology to guide intensification of adjuvant chemotherapy when indicated, and emphasising renal preservation using bil.

这项研究旨在通过加强术前化疗，包括长春新碱，放线菌素-D和多柔比星（VAD），结合化疗后治疗组织学的信息，指导辅助化疗的强化，从而提高BWT患儿的无事件生存率和总生存率，同时最大限度地保留肾脏。指示，并强调使用bil保存肾脏。

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J Clin Oncol. 2022;40:3047–56.Download referencesAuthor informationAuthors and AffiliationsDepartment of Surgery, St. Jude Children’s Research Hospital, Memphis, TN, USAAndrew J. MurphyDivision of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, USAAndrew J.

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