WAGR syndrome and Wilms tumor outcomes

Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 15, 2025 · Captured 2026-09-09.

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Context: Wilms Tumor / Treatment of Wilms Tumor / Treatment of stage V Wilms tumor and those predisposed to developing bilateral Wilms tumor / Treatment of patients with multicentric or bilaterally predisposed unilateral Wilms tumors

The comprehensive International WAGR Syndrome Association survey was used to analyze tumor characteristics, treatment, congenital risk factors, and kidney function in children with WAGR syndrome and Wilms tumors. Of 145 children with WAGR syndrome, 64 developed Wilms tumors (44%).[18]

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Of patients with Wilms tumors, three had relapsed disease and one died. Most patients presented with low-stage disease (stages I–II) and favorable histology.

Chronic kidney disease developed in five children with WAGR syndrome and no Wilms tumors (6.2%) and in 34 children with WAGR syndrome and Wilms tumors (53.1%). Two children with Wilms tumors required hemodialysis, and one child underwent a kidney transplant.

Compared with total nephrectomy, nephron-sparing surgery appeared protective against chronic kidney disease, but it was only used in 37.5% of cases.

By univariate analysis, chronic kidney disease at any stage was associated with complete nephrectomy (P < .0001), chemotherapy duration longer than 12 months, and three-drug therapy. By multivariate analysis, prior nephrectomy was the only significant variable (P = .0002).

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PubMed 38956810 · Original source

Preserved source evidence · Not medical advice