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?
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
Triphasic histology
Risk of WAGR
Bilateral involvement
Increased urine VMA levels
JIPMER 2018