# Impact of Pulmonary Tumor Burden in Favorable Histology Wilms Tumor Outcomes: A Report From the Children's Oncology Group Study AREN053 — GCMD Library

<i>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</i><br><br><b>Purpose:</b> 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.<div><br></div><div><b>Methods:</b> 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.</div><div><br></div><div><b>Results:</b> 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.</div><div><br></div><div><b>Conclusion:</b> 1q gain is a superior prognostic indicator to pulmonary tumor burden in patients with FHWT with pulmonary-only metastasis.</div>

Type: video · 1 min · posted 2026-03-10
Canonical: https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658

## Chapters
- [0:00](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=0) Prognostic Factors in Favorable Histology Wilms Tumor with Lung Metastases

## Statements
- "Dick et al. published a report from a Children's Oncology Group study in the Journal of Clinical Oncology in 2025" — Jill Knepprath (clinical) [0:14](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=14)
- "The study examined factors in patients with favorable histology Wilms tumor and pulmonary metastases and how those factors may affect outcomes" — Jill Knepprath (clinical) [0:19](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=19)
- "Factors studied included the number of metastases, the size of the metastases, and different biological markers" — Jill Knepprath (clinical) [0:30](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=30)
- "There was no difference in survival based on the number of metastases" — Jill Knepprath (clinical) [0:36](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=36)
- "Patients who had a rapid response to chemotherapy with larger nodules showed worse event-free survival" — Jill Knepprath (clinical) [0:41](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=41)
- "Patients with larger nodules and rapid response to chemotherapy showed no changes in overall survival" — Jill Knepprath (clinical) [0:41](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=41)
- "In multivariable models, neither the size nor the number of nodules had any effect on survival" — Jill Knepprath (clinical) [0:49](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=49)
- "1q gain was associated with worse event-free survival" — Jill Knepprath (clinical) [0:55](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=55)
- "1q gain was associated with worse overall survival" — Jill Knepprath (clinical) [0:55](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=55)
- "1q gain is the superior prognostic factor when compared to size and number of nodules in patients with favorable histology Wilms tumor and pulmonary metastases" — Jill Knepprath (clinical) [1:04](https://library.globalcastmd.com/watch/impact-of-pulmonary-tumor-burden-in-favorable-histology-wilms-tumor-outcomes-a-report-from-the-children-s-oncology-group-study-aren053-11658?t=64)

## Transcript
Your patient has a Wilms tumor with favorable histology, but workup shows that they also have lung metastases. How does this change outcomes for your patient? I'm Jill Knerath with Stay Current MD. This is an article you should know. In 2025, Dicketal published a report from a children's oncology group study. In the Journal of Clinical Oncology, they looked at factors in patients with favorable histology wilms and pulmonary metastases and how those may affect outcomes. Factors included the number of mets, the size of the mets, and different biological markers. When it came to the number of the metastases, there was no difference in survival. Patients who had a rapid response to chemo, larger nodules did show a worse event-free survival, but no changes in overall survival. In multi-variable models, neither the size nor the number of nodules had any effect on survival. However, a 1Q gain was associated with worse event-free survival and overall survival. So what is the takeaway? One cue gain is the superior prognostic factor when compared to size and number of nodules in patients with favorable histology Wilms and pulmonary metastases. Do these results surprise you?

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