In which of the following conditions Aniridia and help hyperophy6 are most likely present
Correct Answer: Wilms tumor
Description: Refer Robbins 9/e p479 the percentage of each component is variable (Fig. 10-29A). 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 fibrocytic or myxoid in nature, although skeletal muscle differentiation is not uncom- mon. Rarely, other heterologous elements are identified, including squamous or mucinous epithelium, smooth muscle, adipose tissue, cailage, and osteoid and neurogenic tissue. Approximately 5% of tumors reveal anaplasia, defined as the presence of cells with large, hyperchromatic, pleomorphic nuclei and abnormal mitoses (Fig. 10-29B). The presence of anaplasia correlates with the presence of TP53 mutations and the emergence of resistance to chemotherapy. Recall that p53 elicits pro-apoptotic signals in response to DNA damage (Chapter 7). The loss of p53 function might explain the relative unresponsiveness of anaplastic cells to cytotoxic
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