Wilm’s tumor is associated with all the following except –
Correct Answer: Polycystic kidney
Description: Ans. is 'c' i.e., Polycystic kidney Wilm's tumor o Wilm's 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 wilm's 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. iii)p53. Wilm's tumor is associated with three distinct syndromes : WAGR syndrome Wilm's 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
Category:
Pediatrics
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