Wilm's tumor associated with A/E
Wilm's 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 M/C clinical presentation 2.Hematuria(Extension into renal pelvis) 3.HTN 4.Weight loss. 5.Pain (least common) IOC:CT scan (CECT) CXR (lung metastasis M/C) ref : - Robbins basic pathology . 9th edition .chapter 6 .pg no :- 262 Bailey and Love 27th edition . chapter 76 .pg no 1421.0
Hemihyperophy
Aniridia
Hypeension
Bilateral polycystic kidney disease
Urology