Wilms'tumor with an apparently balanced translocation t(X; 18) resulting in deletion of the WTX gene
The recent description of a new X chromosome tumor suppressor gene, WTX, that is commonly inactivated in Wilms'tumor prompted us to examine the possible involvement of WTX in a case of Wilms'tumor containing an apparently balanced reciprocal translocation between chromosomes X and 18 (t(X;...
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Published in | Genes chromosomes & cancer Vol. 46; no. 10; pp. 909 - 913 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
01.10.2007
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Online Access | Get full text |
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Summary: | The recent description of a new X chromosome tumor suppressor gene, WTX, that is commonly inactivated in Wilms'tumor prompted us to examine the possible involvement of WTX in a case of Wilms'tumor containing an apparently balanced reciprocal translocation between chromosomes X and 18 (t(X; 18)(q11; p11)). Fluorescence in situ hybridization (FISH) analysis of paraffin tumor sections indeed revealed a deletion of the WTX locus at Xq11. High-resolution array comparative genomic hybridization (array CGH) analysis of tumor DNA revealed a 1.5 Mb chromosome deletion encompassing the WTX gene at Xq11. No loss of genetic material was detected on chromosome 18. Interestingly, unlike most tumors with acquired chromosomal translocations, where a new fusion oncogene or promoter-oncogene fusion is created and drives tumor growth, the t(X; 18) in this tumor appears to drive tumorigenesis via deletion of a tumor suppressor. This case demonstrates the importance of array CGH and FISH as adjuncts in tumor cytogenetics and in identifying pathogenic microdeletions in balanced translocations that are not truly balanced. |
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Bibliography: | ObjectType-Article-1 SourceType-Scholarly Journals-1 content type line 23 ObjectType-Feature-2 |
ISSN: | 1045-2257 |
DOI: | 10.1002/gcc.20476 |