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Previous Volume 331:586-590 September 1, 1994 Number 9 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Wilms' tumor is the most common intraabdominal solid tumor of childhood, affecting 1 in 10,000 children worldwide. Treatment includes surgical resection and chemotherapy for virtually all affected children and additional radiotherapy for those with advanced disease or adverse prognostic features. This approach leads to cure rates exceeding 80 percent¹.

Certain features require particular attention in management and also provide a key to understanding the complex genetic pathways involved in the development of this tumor. The occurrence of bilateral Wilms' tumor in approximately 5 to 10 percent of affected children,² dictating kidney-sparing resection, indicates an underlying genetic predisposition. Another peculiar . . . [Full Text of this Article]

The Two-Event Hypothesis

WT1, the Tumor-Suppressor Gene at Chromosome 11p13

Structure and Function

Tissue-Specific Expression and Inactivating Mutations

Dominant Negative Mutations

WT2, the Putative Wilms' Tumor-Suppressor Gene at Chromosome 11p15

Additional Wilms' Tumor Loci

p53 Mutations

Nephrogenic Rests

Future Directions

Source Information

From the Pediatric Oncology Program, Alberta Children's Hospital, Calgary, Alta., and the Department of Oncology, Tom Baker Cancer Center, Calgary, Alta. (M.J.C.); Harvard Medical School, Boston, and the Massachusetts General Hospital Cancer Center, Charlestown, Mass. (D.A.H.); the Department of Pediatrics, Cross Cancer Institute, Edmonton, Alta., and the Faculty of Medicine, University of Alberta, Edmonton (P.E.G.).

Address reprint requests to Dr. Coppes at the Pediatric Oncology Program, Alberta Children's Hospital, 1820 Richmond Rd. S.W., Calgary, AB T2T 5C7, Canada.

References

Appendix

This article has been cited by other articles:

• Bjorge, T., Cnattingius, S., Lie, R. T., Tretli, S., Engeland, A. (2008). Cancer Risk in Children with Birth Defects and in Their Families: A Population Based Cohort Study of 5.2 Million Children from Norway and Sweden. Cancer Epidemiol. Biomarkers Prev. 17: 500-506 [Abstract] [Full Text]  
• Ramburan, A, Oladiran, F, Smith, C, Hadley, G P, Govender, D (2005). Microsatellite analysis of the adenomatous polyposis coli (APC) gene and immunoexpression of {beta} catenin in nephroblastoma: a study including 83 cases treated with preoperative chemotherapy. J. Clin. Pathol. 58: 44-50 [Abstract] [Full Text]  
• Porteus, M. H., Narkool, P., Neuberg, D., Guthrie, K., Breslow, N., Green, D. M., Diller, L. (2000). Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms' Tumor: A Report From the National Wilms Tumor Study Group. JCO 18: 2026-2031 [Abstract] [Full Text]  
• Charles, A. K., Brown, K. W., Berry, P. J. (1998). Microdissecting the Genetic Events in Nephrogenic Rests and Wilms' Tumor Development. Am. J. Pathol. 153: 991-1000 [Abstract] [Full Text]  
• Gicquel, C., Raffin-Sanson, M.-L., Gaston, V., Bertagna, X., Plouin, P.-F., Schlumberger, M., Louvel, A., Luton, J.-P., Le Bouc, Y. (1997). Structural and Functional Abnormalities at 11p15 Are Associated with the Malignant Phenotype in Sporadic Adrenocortical Tumors: Study on a Series of 82 Tumors. J. Clin. Endocrinol. Metab. 82: 2559-2565 [Abstract] [Full Text]  
• Vu, T. H., Hoffman, A. R. (1996). Alterations in the Promoter-specific Imprinting of the Insulin-like Growth Factor-II Gene in Wilms' Tumor. J. Biol. Chem. 271: 9014-9023 [Abstract] [Full Text]