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Clinical Trial
. 2017 Sep;266(3):470-478.
doi: 10.1097/SLA.0000000000002356.

Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group

Peter Ehrlich  1 , Yuen Y Chi, Murali M Chintagumpala, Fred A Hoffer, Elizabeth J Perlman, John A Kalapurakal, Ann Warwick, Robert C Shamberger, Geetika Khanna, Tom E Hamilton, Ken W Gow, Arnold C Paulino, Eric J Gratias, Elizabeth A Mullen, James I Geller, Paul E Grundy, Conrad V Fernandez, Michael L Ritchey, James S Dome
Affiliations
Clinical Trial

Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group

Peter Ehrlich et al. Ann Surg. 2017 Sep.

Erratum in

Abstract

Objective: The Children's Oncology Group study AREN0534 aimed to improve event-free survival (EFS) and overall survival (OS) while preserving renal tissue by intensifying preoperative chemotherapy, completing definitive surgery by 12 weeks from diagnosis, and modifying postoperative chemotherapy based on histologic response.

Background: No prospective therapeutic clinic trials in children with bilateral Wilms tumors (BWT) exist. Historical outcomes for this group were poor and often involved prolonged chemotherapy; on NWTS-5, 4-year EFS for all children with BWT was 56%.

Methods: 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.

Results: 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%).

Conclusion: 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 BWT.

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Conflict of interest statement

The authors have NO conflicts of interest

Figures

Figure 1
Figure 1. Kaplan Meier Curve ARENO534 Event Free Survival and Overall Survival for all patients and histology
See this image and copyright information in PMC

References

    1. Dome JS, Fernandez CV, Mullen EA, et al. Children's Oncology Group's 2013 blueprint for research: renal tumors. Pediatr Blood Cancer. 2013;60(6):994–1000. - PMC - PubMed
    1. Dome JS, Perlman EJ, ML R, et al. Renal Tumors. In: PA P, DG P, editors. Principles and Practice of Pediatric Oncology. Philadelphia: Lippincott Williams and Wilkins; 2015. pp. 753–772.
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    1. Dome JS, Cotton CA, Perlman EJ. Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. J Clin Oncol. 2006;24(15):2352–2358. - PubMed

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