A 5 year old child presents with renal mass. Considering the most common cause in this age group, which of the following is not true?
Correct Answer: Increased urine VMA levels
Description: Wilms tumor in children is MC primary renal tumor of childhood. Peak incidence between 2 and 5 year MC initial clinical presentation for WT is incidental discovery of an asymptomatic abdominal mass by parents while bathing or clothing or by a physician during a routine examination. Morphology: Triphasic combination: Blastemal Stromal Epithelial Congenital Malformation with increased Risk of Wilms tumor WAGR syndrome (33% risk) Denys-Drash syndrome (90% risk, Maximum risk) Q Beckwith-Widemann syndrome (BWS) WT1 gene: Chr 11p13Q Wilms tumor, Aniridia, Genital anomalies, and mental Retardation. Gonadal dysgenesis (male pseudo hermaphroditism) Early-onset nephropathy (diffuse mesangial sclerosis) Increased risk of gonadoblastomasQ Organomegaly: macroglossiaQ, HemihyperophyQ, omphalocele, and abnormal large cells in the adrenal coex (adrenal cytomegaly) Genomic imprintingQ is the causative mechanism. Increased urine VMA levels are seen in Pheochromocytoma
Category:
Pediatrics
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