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 1q chromosomal gain is a stronger predictor of outcomes than pulmonary tumor burden. Treatment stratification based on rapid versus slow nodule response guided therapy intensity including lung radiation.

1q chromosomal gain is a stronger predictor of outcomes than pulmonary tumor burden in stage IV favorable histology Wilms tumor patients.
Rapid complete responders to DD-4A chemotherapy can avoid lung radiation while maintaining good outcomes regardless of initial nodule count.
In slow incomplete responders treated with intensified therapy plus lung RT, initial pulmonary tumor burden does not significantly impact survival.
Early nodule response assessment after 2 cycles of chemotherapy effectively stratifies treatment intensity in metastatic Wilms tumor.
Size of lung metastases showed limited prognostic value compared to molecular markers like 1q gain in this cohort of 251 children.

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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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