ArticlePediatric nephrology (Berlin, Germany)2022
Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant.
Article in Pediatric nephrology (Berlin, Germany), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 17 citations in OpenAlex.
- Wilms tumor characteristics in children with heterozygous germline DIS3L2 variants.Genetics in medicine : official journal of the American College of Medical Genetics · 2025Article
- Comparative Evaluation of Urinary Biomarkers in Wilms Tumor Survivors and Children with Chronic Kidney Disease.International journal of molecular sciences · 2025Article
- Clinical Characterization of a National Cohort of Patients With GermlineKidney international reports · 2024Article
- WT1-related disorders: more than Denys-Drash syndrome.Pediatric nephrology (Berlin, Germany) · 2024Review
- Most appropriate surgical approach in children with Wilms tumour, risk of kidney disease, and related considerations.Pediatric nephrology (Berlin, Germany) · 2024Article
- Hypertension in Wilms tumor.Pediatric nephrology (Berlin, Germany) · 2024Review
- Prechemotherapy Transperitoneal Robotic-Assisted Partial Nephrectomy (RAPN) for a Wilms Tumor: Surgical and Oncological Outcomes in a Four-Year-Old Patient.Pediatric reports · 2023Article
- Spectrum of Clinical Manifestations in Children WithFrontiers in pediatrics · 2022Article
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14 authors at 3 institutions in 3 countries.
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Abstract
backgroundWilms tumour (WT) survivors, especially patients with associated syndromes or genitourinary anomalies due to constitutional WT1 pathogenic variant, have increased risk of kidney failure. We describe the long-term kidney function in children with WT and WT1 pathogenic variant to inform the surgical strategy and oncological management of such complex children.
methodsRetrospective analysis of patients with WT and constitutional WT1 pathogenic variant treated at a single centre between 1993 and 2016, reviewing genotype, phenotype, tumour histology, laterality, treatment, patient survival, and kidney outcome.
resultsWe identified 25 patients (60% male, median age at diagnosis 14 months, range 4-74 months) with WT1 deletion (4), missense (2), nonsense (8), frameshift (7), or splice site (4) pathogenic variant. Thirteen (52%) had bilateral disease, 3 (12%) had WT-aniridia, 1 had incomplete Denys-Drash syndrome, 11 (44%) had genitourinary malformation, and 10 (40%) had no phenotypic anomalies. Patient survival was 100% and 3 patients were in remission after relapse at median follow-up of 9 years. Seven patients (28%) commenced chronic dialysis of which 3 were after bilateral nephrectomies. The overall kidney survival for this cohort as mean time to start of dialysis was 13.38 years (95% CI: 10.3-16.4), where 7 patients experienced kidney failure at a median of 5.6 years. All of these 7 patients were subsequently transplanted. In addition, 2 patients have stage III and stage IV chronic kidney disease and 12 patients have albuminuria and/or treatment with ACE inhibitors. Four patients (3 frameshift; 1 WT1 deletion) had normal blood pressure and kidney function without proteinuria at follow-up from 1.5 to 12 years.
conclusionsDespite the known high risk of kidney disease in patients with WT and constitutional WT1 pathogenic variant, nearly two-thirds of patients had sustained native kidney function, suggesting that nephron-sparing surgery (NSS) should be attempted when possible without compromising oncological risk. Larger international studies are needed for accurate assessment of WT1genotype-kidney function phenotype correlation.
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