<prompt> Wilm s tumor associated with AEHemihypertrophyAniridiaHypertensionBilateral polycystic kidney disease  <response>: The genitourinary anomalies most commonly associated with Wilms tumor arehypoplasia,fusion and ectopia of the kidney,duplications of the collecting systems,hypospadias, andcryptorchidismBilateral polycystic kidney disease is not associated with the Wilms tumorSyndromes Associated with Wilms TumorSeveral syndromes and congenital abnormalities commonly are reported in patients with Wilms tumor.WAGR syndrome contiguous gene deletion of WT1 PAX6 on 11P13It consists ofWilms tumor,Aniridia,Genitourinary abnormalities cryptorchidism, streak ovaries, bicornuate uterus, ambiguous genitalia, andMental retardation.Patients with this syndrome have a constitutional deletion of chromosome 11p13 where the Wilms tumor gene,WT1, and the aniridia gene, PAX6, are located.Denys- Drash syndromemissense mutation in WT1 gene It is characterized byMale pseudohermaphrodism,Early-onset renal failure characterized by mesangial sclerosis andIncreased risk of Wilms tumor.Patients with this syndrome typically carry a missense mutation in the WT1 gene.Beckwith-Wiedemann syndrome WT2 gene abnormality on chromosome 11P15.5It is characterized byHemihypertrophy,Macroglossia,Visceromegaly,OmphaloceleRisk of developing Wilms tumor 3-5A variety of 11p15.5 abnormalities have been reported in patients with this syndrome, and it is postulated that a second Wilms tumor gene, WT2, is located in this region. Loss of imprinting of the insulin-like growth factor 2 gene, an epigenetic process, also is associated with Wilms tumor.Other syndromes or conditions with an increased risk of Wilms tumor include:Hemihypertrophy,Sporadic aniridia,Genitourinary anomalies,Pearlman syndrome,Sotos syndrome,Neurofibromatosis von Recklinghausen disease, and Von Willebrand disease.About option c Hypertension.Hypertension also has been described and probably is due to renal ischemia.- NelsonMore about Wilms tumor also ka nephroblastoma:Wilms tumor is the most common renal neoplasm 2nd most common malignant abdominal tumor, in children Most common abdominal malignant tumor is neuroblastomaPeak incidence is between 2 and 5 yrs of age.5-10 are bilateral; either both kidneys are involved simultaneously synchronous or one after the other metachronousClinical features of W.T.Abdominal lump most common presenting featureAbdominal painVomitingHematuriaHypertensionTreatment:High cure rates of 80-90 can be achieved with multimodality treatment using surgery, chemotherapy radiotherapySurgical resection is done in all stages followed by chemotherapy stage III or chemotherapy radiotherapy stage III, IV, VPre op chemotherapy is given in inoperable bilateral casesChemotherapeutic agents used are Vincristine, Dactinomycin, Doxorubicin Cyclophosphamide. 