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Eur J Cancer. 2019 Sep;118:58-66. doi: 10.1016/j.ejca.2019.05.033. Epub 2019 Jul 17.

Treatment of stage I anaplastic Wilms' tumour: a report from the Children's Oncology Group AREN0321 study.

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Division of Pediatrics, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 87, Houston, TX 77030-4009, USA. Electronic address:
Department of Biostatistics, University of Florida, 6011 NW 1st Place Suite 10, Gainesville, FL 32607, USA. Electronic address:
Department of Biostatistics, University of Florida, 6011 NW 1st Place Suite 10, Gainesville, FL 32607, USA.
Department of Pediatric Hematology/Oncology, Dana-Farber/Harvard Cancer Center, Dana Farber Cancer Institute, 450 Brookline Ave. Office#: SW360A, Boston, MA 02215-5450, USA. Electronic address:
Department of Radiation Oncology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern Memorial Hospital, 25i East Huron Street Suite#L178, Chicago, IL 60611, USA. Electronic address:
Children's Oncology Group Data Center, Statistics Data Center, 6011 NW 1st Place Suite 10, Gainesville, FL 32607, USA. Electronic address:
Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S. Kingshighway, Box 8131, St. Louis, MO 63110, USA. Electronic address:
Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Avenue MLC 7015, Cincinnati, OH 45229, USA. Electronic address:
Department of Pathology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 East Chicago Avenue, Chicago, IL 60611, USA. Electronic address:
Department of Pediatric Surgery, University of Michigan, 4-945 C.S. Mott Children's Hospital SPC 4211, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-4211, USA. Electronic address:
Department of Pediatrics, F. Edward Hébert School of Medicine, Uniformed Services University, 4301 Jones Bridge Road, Bethesda, MD 20814, USA. Electronic address:
Department of Pediatrics, University of Alberta Hospital, WMC 4E2.28, 8440-112 Street, Edmonton, Alberta T6G 2B7, Canada. Electronic address:
Departments of Pediatrics and Bioethics, IWK Health Centre, Dalhousie University and the IWK Health Centre, 5850 University Avenue, Halifax, Nova Scotia B3K 6R8, Canada. Electronic address:
Division of Oncology, Children's National Medical Center, Center for Cancer and Blood Disorders, George Washington University School of Medicine and Health Sciences, 111 Michigan Avenue NW, Washington, DC 20010, USA. Electronic address:



In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free survival (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n = 10) or diffuse (n = 19) anaplastic Wilms' tumour (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5% and 82.6%, respectively. The Children's Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients.


Tumour histology and stage were confirmed by real-time central pathology, surgery and radiology review. The patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analysed outcomes of all patients with stage I AWT enrolled in NWTSs 1-5 and AREN0321 with respect to treatment regimens.


Eighteen patients with stage I AWT (8 focal and 10 diffuse) were enrolled on AREN0321. With a median follow-up of 4.6 years, the 4-year EFS and OS were 100%. One patient with diffuse AWT had pulmonary relapse 4.12 years after diagnosis. In the 112 patients with stage I AWT treated in NWTSs 1-5 and AREN0321, the EFS was significantly improved with doxorubicin treatment (p = 0.01; 4-year EFS: 97.2% [95% confidence interval {CI}: 91.3-100] vs. 77.5% [95% CI: 67.6-87.4]) but not by flank radiation (p = 0.15).


Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. Retrospective analysis of AREN0321 and NWTS patients suggests that doxorubicin had a greater contribution to the excellent outcomes than radiation.


Dactinomycin; Diffuse anaplasia; Doxorubicin; Focal anaplasia; Outcome; Radiation; Stage I; Treatment; Vincristine; Wilms tumour

[Available on 2020-09-01]

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