/Wilms tumor 1

Wilms tumor 1

Rare Disease

MONDO:0008679

Also Known As

Wilms' tumorWilms' tumourrenal Wilms tumorrenal Wilms tumourWT1-related Wilms tumor predispositionWilms tumor 1Wilms tumor type 1Wilms tumor, autosomal dominant, somatic mutationWilms tumor, somaticWilms tumor, type 1, autosomal dominant, somatic mutationWilms tumour type 1WT1nephroblastoma

Definition

An autosomal disorder due to pathogenic variants in the WT1 gene leading to an increased risk Wilms tumor and genitourinary abnormalities incorporating Denys-Drash syndrome and Frasier syndrome.