A child presents with abdominal mass. Biopsy showed a triphasic tumour with areas of small blue cells. Which of the following is a feature of this tumour?
Correct Answer: WT1 mutation
Description: Small blue round tumour cells seen in- retinoblastoma, medulloblastoma, Ewing sarcoma, neuroblastoma, Wilms tumor, ALL Microscopically, Wilms tumors are characterized by recognizable attempts to recapitulate different stages of nephrogenesis. The classic triphasic combination of blastemal, stromal, and epithelial cell types is observed in the majority of Wilms tumours. Sheets of small blue cells with few distinctive features characterize the blastemal component. Epithelial differentiation is usually in the form of aboive tubules or glomeruli. Stromal cells are usually fibroblastic or myxoid in nature. Nephrogenic rests are putative precursor lesions of Wilms tumors and are seen in the renal parenchyma adjacent. WT1 protein is critical for normal renal and gonadal development. Wilms tumors demonstrate WT1 mutations. MYCN amplification - neuroblastoma C-MYC overexpression - Burkitt lymphoma RB deletion - retinoblastoma
Category:
Pathology
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