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File:IPLab13WT10.jpg|This high-power photomicrograph shows the differences in cell morphology between the blastema (1) and the fibroblast type cells (2).
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== Study Questions ==
* <spoiler text="What genetic factors predispose to the development of Wilms' tumors?">There are at least three known congenital malformations with defects in at least two chromosomal loci which predispose to Wilms' tumor.
The first group has the WAGR syndrome characterized by aniridia, genital anomalies, and mental retardation and a 33% chance of developing Wilms’ tumor. These patients have a gene defect proximal to chromosome 11 band p13 which is the Wilms’ tumor-associated gene, WT-1.
A second group of patients at risk for Wilms’ tumor have the Denys-Drash syndrome, which is characterized by gonadal dysgenesis (male pseudohermaphroditism) and nephropathy leading to renal failure. The majority of these patients develop Wilms’ tumors. The genetic abnormality in these children has also been mapped to chromosome 11, band p13.
The third group of patients at increased risk of developing Wilms’ tumor are those children with Beckwith-Wiedemann syndrome. This syndrome is characterized by enlargement of body organs, hemihypertrophy, renal medullary cysts, and abnormal large cells in adrenal cortex (adrenal cytomegaly). The genetic locus that is involved in these patients is in band p15.5 of chromosome 11 distal to the WT-1 locus.
The function of the second Wilms’ tumor gene (WT-2) is unknown. A few familial cases of Wilms’ tumors not associated with identifiable deletions or mutations involving either the WT-1 or the WT-2 gene suggest that there may be another locus that plays a role in some tumors.</spoiler>
* <spoiler text="What are the usual presenting signs with Wilms' tumor and what is the prognosis?">Children with Wilms’ tumors usually present with a large abdominal mass.
Hematuria, pain in the abdomen following some hemorrhagic incident, intestinal obstruction, and hypertension are other patterns of presentation.
With chemotherapy, radiotherapy, and surgery there is a 90% long-term survival rate.</spoiler>
{{IPLab 13}}
[[Category: IPLab:Lab 13]]
