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Telomere fusion as a mechanism for the progressive loss of the short arm of chromosome 11 in an anaplastic wilms' tumor
Author(s) -
Sawyer Jeffrey R.,
Goosen Linda S.,
Stine Kimo C.,
Thomas James R.
Publication year - 1994
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19940715)74:2<767::aid-cncr2820740232>3.0.co;2-f
Subject(s) - loss of heterozygosity , telomere , chromosomal translocation , chromosome , chromosome instability , genetics , wilms' tumor , biology , karyotype , cancer research , allele , dna , gene
Wilms' tumor (WT) is associated with chromosomal deletions and loss of heterozygosity (LOH) of alleles at 11p13. The authors report the youngest known patient with diffusely anaplastic WT and, to their knowledge, the first case to demonstrate telomeric fusions as a chromosomal mechanism for the loss of bands 11p13 and 11p15 in WT. Recurrent clonal telomeric association (tas) initiated breakage/fusion cycles that resulted in deletions of chromosome bands 11p15, 11p13, and subsequently, the entire short arm of chromosome 11. In addition, tas involving the long arms of chromosomes 7 and 9 resulted in the subsequent deletion of the long arm of chromosome 7. This report expands the spectrum of chromosomal mechanisms that can account for the loss of alleles on the short arm of chromosome 11 in WT by providing evidence that the progressive loss of critical chromosome regions associated with tumor suppression may occur as a result of chromosomal instability initiated by tas. Cancer 1994; 74: 767‐73.