Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant.

ABSTRACT:

Background

Wilms 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.

Methods

Retrospective 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.

Results

We identified 25 patients (60% male, median age at diagnosis 14 months, range 4-74 months) with WT1 deletion (4), missense (2), nonsense (8), fra

SUBMITTER: Falcone MP 

PROVIDER: S-EPMC8960606 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature