AREN0534 | Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor

DOI: 10.7937/TCIA.5M9S-6Y97 | Data Citation Required | Image Collection

Summary

This collection contains data from the Children’s Oncology Group (COG) Clinical Trial NCT00945009, “Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor, " Study Chair: Peter F. Ehrlich, M.D. M.S.C. It was sponsored by NCI and performed by the Children's Oncology Group under study number AREN0534. This phase III trial studies how well combination chemotherapy and surgery work in treating young patients with Wilms tumor. Select patient-level clinical data from this trial is available via the following link: https://nctn-data-archive.nci.nih.gov/node/728.

Trial Description

Children with bilateral Wilms tumor account for 5-7% of all patients with Wilms tumor. Certain patients, with syndromes associated with Wilms tumor development, have been identified to be at increased risk for bilateral tumors. Due to an increased risk for renal failure, patients with bilateral disease at presentation are treated with preoperative chemotherapy in order to preserve renal parenchyma. Although this recommendation was made nearly 30 years ago, patients with bilateral tumors have not been formally studied in prior cooperative trials. Recent evidence suggests that survival of these patients is inferior to similar patients with unilateral tumor. This study is designed to improve the survival of these children, while continuing the emphasis on preserving renal function. Patients were enrolled and imaging studies were centrally reviewed to assess for bilateral renal lesions. They were treated with 3-drug induction chemotherapy (vincristine, dactinomycin, and doxorubicin) for 6 or 12 weeks based on radiographic response followed by surgery and further chemotherapy determined by histology. Radiation therapy was provided for postchemotherapy stage III and IV disease. One hundred eighty-nine of 208 patients were evaluable. Four-year EFS and OS were 82.1% (95% CI: 73.5%–90.8%) and 94.9% (95% CI: 90.1%–99.7%. Twenty-three patients relapsed and 7 had disease progression. After induction chemotherapy 163 of 189 (84.0%) underwent definitive surgical treatment in at least 1 kidney by 12 weeks and 39% retained parts of both kidneys. Surgical approaches included: unilateral total nephrectomy with contralateral partial nephrectomy (48%), bilateral partial nephrectomy (35%), unilateral total nephrectomy (10.5%), unilateral partial nephrectomy (4%), and bilateral total nephrectomies (2.5%). This treatment approach including standardized 3-drug preoperative chemotherapy, surgical resection within 12 weeks of diagnosis and response and histology-based postoperative therapy improved EFS and OS and preservation of renal parenchyma compared with historical outcomes for children with bilateral Wilms tumor.

Before enrollment, real-time central review of diagnostic imaging, pathology (if obtained), and operative notes confirmed the status of BWT. After 6 weeks, cross-sectional imaging was performed and a tumor response was assigned for each kidney. After 4 chemotherapy cycles (12 weeks), repeat cross sectional imaging was performed and definitive surgery was required. Response was based on the Response Evaluation Criteria in Solid Tumor (RECIST 1.1) modified to include 3 lesions per kidney.

Trial Outcomes

Results of the trial have been reported in the following publication:

• Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS. Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group. Ann Surg. 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. Erratum in: Ann Surg. 2018 Mar;267(3):e64. PMID: 28795993; PMCID: PMC5629006.
• Ehrlich PF, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Tornwall B, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Dome JS. Results of Treatment for Patients With Multicentric or Bilaterally Predisposed Unilateral Wilms Tumor (AREN0534): A report from the Children's Oncology Group. Cancer. 2020 Aug 1;126(15):3516-3525. doi: 10.1002/cncr.32958. Epub 2020 May 27. PMID: 32459384; PMCID: PMC7769115.

Data Access

This is a limited access data set. To request access please register an account on the NCTN Data Archive. After logging in, use the “Request Data” link in the left side menu. Follow the on screen instructions, and enter NCT00945009 when asked which trial you want to request. In step 2 of the Create Request form, be sure to select “Imaging Data Requested”. Please contact NCINCTNDataArchive@mail.nih.gov for any questions about access requests.

Version 1: Updated 2021/11/09

Additional Resources for this Dataset

The National Cancer Institute (NCI) has created a centralized, controlled-access database, called the NCTN/NCORP Data Archive, for storing and sharing datasets generated from clinical trials of the National Clinical Trials Network (NCTN) and the NCI Community Oncology Research Program (NCORP). Clinical data from the participants in this trial can be found at:

• NCTN/NCORP Data Archive (Clinical Data)

Citations & Data Usage Policy

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