Impact of Pulmonary Tumor Burden in Favorable Histology Wilms Tumor Outcomes: A Report From the Children's Oncology Group Study AREN0533

Infographic · Feb 2026 · 1 min read

In brief

COG study AREN0533 analyzed 251 children with favorable histology Wilms tumor and lung-only metastases, finding that chromosomal 1q gain was a stronger predictor of outcomes than pulmonary tumor burden. Rapid responders avoided lung radiation while maintaining comparable survival to those requiring intensified therapy.

1q gain is a stronger prognostic indicator than pulmonary tumor burden in stage IV favorable histology Wilms tumor with lung-only metastases.
Rapid complete responders to DD-4A chemotherapy can avoid lung radiation without compromising outcomes regardless of initial nodule count.
Size of pulmonary metastases correlates with event-free survival in rapid responders but not in slow/incomplete responders receiving intensified therapy.
Number of lung nodules does not significantly predict outcomes in either rapid or slow responders when stratified by treatment response.
Slow responders require intensified chemotherapy (Regimen M) plus lung radiation regardless of initial pulmonary tumor burden.

Written by the GCMD Library team from the infographic.

The infographic uses a teal header with lung illustration on the left. Two treatment response groups are shown in contrasting boxes: green for Rapid Complete Responders and magenta for Slow Incomplete Responders. A DNA helix icon and kidney illustration flank the central predictor section. The bottom features a yellow conclusion banner with institutional logos and citation.

David B Dix, Geetika Khanna, Lindsay A Renfro, Ian C Tfirn, Ethan A Smith, Maddy Artunduaga, Meryle J Eklund, Jesse K Sandberg, Lauren N Parsons, John A Kalapurakal, Peter F Ehrlich, Jennifer H Aldrink, Richard D Glick, Daniel J Benedetti, Conrad V Fernandez, Jeffrey S Dome, Elizabeth A Mullen, James I Geller; COG Renal Tumor Committee

Abstract

Purpose: Children with favorable histology Wilms tumor (FHWT) with pulmonary metastases have inferior outcomes compared with those with localized disease. We evaluated the impact of pulmonary tumor burden within subgroups of similarly treated children with stage IV FHWT.

Methods: Children with FHWT with pulmonary-only metastasis enrolled in AREN0533 were included. Lung nodule response assessment, by chest computed tomography after two cycles of vincristine/dactinomycin/doxorubicin (Regimen DD-4A) chemotherapy, identified rapid complete responses (RCRs) and slow incomplete pulmonary nodule responses (SIRs). Event-free survival (EFS) and overall survival (OS) were compared by number and size of pulmonary metastases within two cohorts: (1) RCR treated with additional DD-4A without lung radiation therapy (RT) and (2) SIR treated with the original three drugs plus cyclophosphamide/etoposide (Regimen M) with lung RT. The multivariable Cox proportional hazards model for EFS and OS stratified by treatment assessed the impact of the number and size of pulmonary metastases adjusted for tumor 1q gain.

Results: AREN0533 enrolled 288 children with stage IV pulmonary-only metastases, of whom 251 met inclusion criteria for outcome analyses. In the RCR cohort (n = 105), EFS and OS were not significantly different based on the number of lung metastases, whereas size of pulmonary metastases was significantly associated with EFS (P = .022), but not OS. In the SIR cohort (n = 146), EFS and OS did not differ by the number or size of lung metastases. In multivariable models, neither number nor size of lung metastases was significantly associated with EFS or OS although 1q gain was significant (EFS P = .0015; OS P = .039) after adjustment for these factors.

Conclusion: 1q gain is a superior prognostic indicator to pulmonary tumor burden in patients with FHWT with pulmonary-only metastasis.

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