<prompt> Wilms tumor associated with AEHemihyperophyAniridiaHypeensionBilateral polycystic kidney disease  <response>: Wilms tumour nephroblastoma is a mixed tumour containing blastemal, stromal and epithelial elements arising from embryonic nephrogenic tissue. It is the most common primary tumour of kidney in children . Most commonly between 2 and 5 years of age . It is associated with three congenital malformation syndromes 1 WAGR SYNDROME . wilms tumour , ANIRIDIA , mental retardation 2 DENYS - DRASH SYNDROME . gonadal dysgenesis and renal abnormalities. These two syndromes are associated with WT1 mutations. 3 BECKWITH-WIDEMANN SYNDROME is associated with Macroglossia,Visceromegaly, enlargement of entire segment HEMI HYPEROPHY , enlargement of adrenal coical cells adrenal cytomegaly is charecteristic microscopic feature.Midline abdominal wall defect .BWS is an example of disorder of genomic imprinting. CLINICL FEATURES :- 1.Flank mass MC clinical presentation 2.HematuriaExtension into renal pelvis 3.HTN 4.Weight loss. 5.Pain least common IOC:CT scan CECT CXR lung metastasis MC ref : - Robbins basic pathology . 9th edition .chapter 6 .pg no :- 262 Bailey and Love 27th edition . chapter 76 .pg no 1421.0 