<prompt> Wilms tumor is associated with all the following except -AniridiaBeckwith syndromePolycystic kidneyHemi-hyperohy  <response>: Ans. is c i.e., Polycystic kidney Wilms tumor o Wilms tumor, also known as nephroblastoma, is the most common malignant tumor of kidney in children. Etiology o Most of the tumor are sporadic. o 1 - 2 may have family history. o Familial predisposition to wilms tumor is inherited in an autosomal dominant manner. o Mutation of the following genes are seen in wilms tumor. i Wilms tumor gene WT I on chromosome 11 ii CTNNB1 gene encoding the protooncogene beta-catenin. iiip53. Wilms tumor is associated with three distinct syndromes : WAGR syndrome Wilms tumor Aniridia Genital abnormalities Mental Retardation Beckwith Wiedemann syndrome Enlargement of body organs Hemihyperophy Renal medullary cysts 3 Adrenal cytomegaly : abnormal large cells in adrenal coex Denys-Drash syndrome 3 Gonadal dysgenesis Male pseudo-hermaphrodite o Nephropathy leading to Renal failure 