This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Surgery
[{'content': "Wilm' s tumor associated with A/E \nSelect and explain. \nA. Hemihypertrophy, B. Aniridia, C. Hypertension, D. Bilateral polycystic kidney disease", 'role': 'user'}, {'content': 'Answer: D \nExplanation: The genitourinary anomalies most commonly associated with Wilms tumor are\n\nhypoplasia,\nfusion and ectopia of the kidney,\nduplications of the collecting systems,\nhypospadias, and\ncryptorchidism\n(Bilateral polycystic kidney disease is not associated with the Wilm\'s tumor)\n\nSyndromes Associated with Wilms Tumor\n\nSeveral syndromes and congenital abnormalities commonly are reported in patients with Wilms tumor.\nWAGR syndrome (contiguous gene deletion of WT1 & PAX6 on 11P13)\nIt consists of\nWilms tumor,\nAniridia,\nGenitourinary abnormalities (cryptorchidism, streak ovaries, bicornuate uterus, ambiguous genitalia), and\nMental retardation.\nPatients with this syndrome have a constitutional deletion of chromosome 11p13 where the Wilms tumor gene,\nWT1, and the aniridia gene, PAX6, are located.\nDenys- Drash syndrome(missense mutation in WT1 gene)\xa0\nIt is characterized by\nMale pseudohermaphrodism,\nEarly-onset renal failure characterized by mesangial sclerosis and\nIncreased risk of Wilms tumor.\nPatients with this syndrome typically carry a missense mutation in the WT1 gene.\nBeckwith-Wiedemann syndrome (WT2 gene abnormality on chromosome 11P15.5)\nIt is characterized by\nHemihypertrophy,\nMacroglossia,\nVisceromegaly,\nOmphalocele\nRisk of developing Wilms tumor (3-5%)\nA 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.\n\n\nOther syndromes or conditions with an increased risk of Wilms tumor include:\n\nHemihypertrophy,\nSporadic aniridia,\nGenitourinary anomalies,\nPearlman syndrome,\nSotos syndrome,\nNeurofibromatosis (von Recklinghausen disease), and Von Willebrand disease.\n\nAbout option \'c\' Hypertension.\n"Hypertension also has been described and probably is due to renal ischemia."- Nelson\nMore about Wilm\'s tumor (also k/a nephroblastoma):\n\nWilm\'s tumor is the most common renal neoplasm & 2nd most common malignant abdominal tumor, in children (Most common abdominal malignant tumor is neuroblastoma)\nPeak incidence is between 2 and 5 yrs of age.\n5-10% are bilateral; either both kidneys are involved simultaneously (synchronous) or one after the other (metachronous)\n\nClinical features of W.T.\n\nAbdominal lump (most common presenting feature)\nAbdominal pain\nVomiting\nHematuria\nHypertension\n\nTreatment:\n\nHigh cure rates of 80-90% can be achieved with multimodality treatment using surgery, chemotherapy & radiotherapy\nSurgical resection is done in all stages followed by chemotherapy (stage I&II) or chemotherapy + radiotherapy (stage III, IV, V)\nPre op chemotherapy is given in inoperable & bilateral cases\n\nChemotherapeutic agents used are Vincristine, Dactinomycin, Doxorubicin & Cyclophosphamide.', 'role': 'assistant'}]