All of the statements regarding Wilm's tumor are true EXCEPT:
WILM'S TUMOR: *Also known as nephroblastoma *Most tumors are sporadic, but familial predisposition may be autosomal dominant. It most commonly occur in children between 2 and 5 years of age. *Wilms tumor gene (WT 1 on ch 11), p53, CTNNB1 encoding beta catenin Presentation *Asymptomatic abdominal mass (m.c) *Abdominal pain *Hematuria *Hypeension *Fever *Anorexia *Age: usual age is 2-3 years *Poor prognostic factors: Unourable histology, Hyperploidy, Large tumor and Advanced stage (II, IV.) *Metastasis: Usually to lung (mc) and then to liver, Bone metastasis are rare Associated with three syndromes WAGR syndrome (wilm's tumor, aniridia , genital abnormalities , mental retardation) Beckwith wiedmann syndrome (enlargement of body organs, hemihyperophy, renal medullary cysts, adrenal cytomegaly) Denys-Drash syndrome (gonadal dysgenesis i.e. male pseudo hermaphroditism, nephropathy leading to renal failure)
Presents after 5 years of age
Hematuria is the presenting symptom
Presents as abdominal mass
Most commonly metastasize to lung