CONTEXT Mutations of the p53 tumor suppressor gene are the most frequent alterations observed in human neoplasias affecting adults. In pediatric oncology, however, they have seldom been identified. Wilms' tumor is a renal neoplasia commonly occurring in children and is associated with mutations of the WT1 gene. The correlation between Wilms' tumor and alterations of the p53 gene has not been we...

Wilms' tumor is the most common childhood renal malignancy. A genome-wide association study identified LIM domain only 1 (LMO1) as having oncogenic potential. We examined the associations between LMO1 gene polymorphisms and susceptibility to Wilms' tumor. In this hospital-based, case-control study, we recruited 145 children with Wilms' tumor and 531 cancer-free children. Four polymorphisms (rs1...

Wilms' tumor is one of the most prevalent pediatric malignancies, ranking fourth in childhood cancer worldwide. TP53 is a critical tumor suppressor gene, which encodes a 53 kDa protein, p53. The p53 functions to protect against cancer by regulating cell cycle and apoptosis and maintaining DNA integrity. TP53 gene is highly polymorphic. Several TP53 gene polymorphisms have been considered to be ...

Wilms tumor is an embryonal kidney cancer that affects one in 10 000 children. Epidemiologic studies have shown that 1%–3% of cases of Wilms tumor are familial and that a predisposition to Wilms tumor is probably caused by rare germline mutations acting in a dominant fashion (1). The risks of Wilms tumor conferred by mutations in these genes are poorly characterized, with estimates of their pen...

On the search for a genetic cause 46,XX virilization due to testicular (TDSD) or ovotesticular DSD (OTDSD), 78 individuals were studied by whole exome sequencing. In 7 cases, heterozygous de novo variants found in 4th zinc finger (ZF4) of Wilms tumor 1 gene (WT1). Modelling these human granulosa cells and mice recapitulated phenotype.

Mutations of the p53 tumor suppressor gene occur frequently in a variety of adult-onset tumors, including colon, breast, lung, and brain, yet are infrequently identified in pediatric malignancies. Wilms' tumor, a common solid tumor of childhood, can be associated with mutations of the WT1 gene. Alterations of the p53 gene have been shown to modulate the ability of WT1 to transactivate its targe...

We have examined the pattern of expression of the human PAX2 gene in Wilms' tumors and human fetal kidney by Northern blot and in situ hybridization. Human PAX2 encodes a paired box-containing protein and has a high degree of homology with mouse and Drosophila paired box genes. In situ hybridization analysis reveals that PAX2 is expressed in nephrogenic structures in fetal kidney and also in Wi...

Genetic alterations in tumor suppressor genes are believed to play an important role in the initiation of childhood and adult malignancies. Tumor-specific loss of heterozygosity for particular chromosomal regions has provided the starting point for the cloning of different tumor suppressor genes, including the Wilms tumor predisposing gene, WT1, at chromosome 11p13. This article reviews the pat...

background: wilms' tumor is an emberyonal tumor arising from remnants of immature renal tissue. her2/neu is an onco-protein which mediates cellular proliferation, differentiation and survival. methods: in the current study, we analyzed her2/neu expression in 40 wilms' tumors. the clinico-demographic data of 40 patients with wilms' tumor were retrieved. immunohistochemical staining for her2/neu ...