Wilm's tumor is associated with all the following except :
High-Yield Explanation
Warn' s tamer :
Wilm's tumor, also known as nephroblastoma, is the most common malignant tumor of kidney in children. Etiology.
Most of the tumor are sporadic.
1- 2% may have family history.
Familial predisposition to wilm's tumor is inherited in an autosornal dominant manner.
Mutation of the following genes are seen in wilms tumor.
Wilms tumor gene (WT 1) on chromosome 11.
CTNNB I gene encoding the protooncogene beta - ca ten in.
p53.
Wilm's tumor is associated with three distinct syndromes.
WAGR syndrome
Beck with Wiedemnn syndrome
Denys - Drash syndrome
Wilm's tumor
Aniridia
Genital abnormalities
Mental Retardation
Enlargement of body organs
Hemihypertrophy
Renal medullary cysts
Adrenal cytomegaly : abnormal large cells in adrenal cortex
Gonadal dysgenesis (Male pseudo - hermaphrodite)
Nephropathy leading to Renal failure