What question did this study set out to answer?

This review aims to summarize the prognostic value of molecular biomarkers in Wilms tumors, particularly in surgery and preoperative chemotherapy settings.

May 20, 2026JCO Precision Oncology0 citations

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Systematic Review: Prognostic Molecular Biomarkers in Wilms Tumors

AOAgustina Oller PKPatrick Kemmeren DPDaniela Perotti

Key Points

This review aims to summarize the prognostic value of molecular biomarkers in Wilms tumors, particularly in surgery and preoperative chemotherapy settings.
Conducted a systematic literature review including studies with ≥50 de novo Wilms tumors from PubMed and Embase up to January 2025.
Evaluated various biomarkers such as 1q gain and copy number variations in relation to survival outcomes.
Assessed risk of bias using the quality in prognosis studies tool.
1q gain was identified as a worse prognostic factor for event-free survival (EFS) in up-front nephrectomy with low bias analyses.
1p and/or 16q loss of heterozygosity (LOH/loss) were noted as independent adverse prognostic factors for EFS and overall survival (OS).
Adverse prognostic implications of TP53 and MYCN were observed in univariate analyses, while other biomarkers showed limited significant data.

Abstract

PURPOSE Molecular biomarkers are increasingly used for risk stratification, particularly in up-front surgery settings (Children's Oncology Group trials), whereas in preoperative chemotherapy setting, the ongoing International Society of Pediatric Oncology (SIOP)-Renal Tumor Study Group-2016 UMBRELLA study aims to validate selected biomarkers for future risk-adapted treatment strategies. This systematic review summarizes all literature on the prognostic value of these biomarkers. MATERIALS AND METHODS A systematic literature review (PubMed and Embase; up to January 2025) included studies with ≥50 de novo Wilms tumors (WTs). Eligible biomarkers included copy number variations; 1q gain, 1p and/or 16q loss of heterozygosity (LOH)/loss, 12 gain, 14q loss, 22 loss, 11p15 LOH/loss of imprinting (LOI), and structural somatic variants ( TP53 and/or 17p loss, MYCN , FBXW7 , WT1 , WTX , SIX1/SIX2 , DROSHA , DGCR8 , AMER1 , CTNNB1 , GPC3 , MLLT1 , DICER1 , DIS3L2 ). Outcome included relapse-free survival, event-free survival (EFS), and overall survival (OS). Risk of bias was assessed with quality in prognosis studies tool. RESULTS Low-bias multivariable/stratified analyses identified 1q gain as worse EFS and 1p and/or 16q LOH/loss as worse EFS/OS prognostic factors, in up-front nephrectomy settings. Preoperative chemotherapy settings revealed similar trends with lacking significance. TP53 and MYCN were adverse prognostic in univariate analyses. No prognostic data were available for the remaining variants. CONCLUSION 1q gain and 1p and/or 16q LOH/loss emerge as independent prognostic biomarkers in up-front nephrectomy settings. Evidence remains limited in preoperative chemotherapy settings, particularly when using SIOP-oriented treatment algorithms. Prognostic value of TP53 , MYCN , and 11p15 LOH/LOI warrants further validation in both settings. This highlights the need for adequately powered prospective studies, specifically in the preoperative chemotherapy setting, to establish reliable molecular biomarkers.

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Cite This Study

Oller et al. (Fri,) studied this question.

synapsesocial.com/papers/6a0d50f3f03e14405aa9d124 https://doi.org/https://doi.org/10.1200/po-25-01017

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