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High-resolution genomic profiling of an adult Wilms' tumor: evidence for a pathogenesis distinct from corresponding pediatric tumors.

Karlsson, Jenny LU ; Holmquist Mengelbier, Linda LU ; Elfving, Peter LU and Gisselsson Nord, David LU (2011) In Virchows Archiv: an international journal of pathology 459. p.547-553
Abstract
Wilms' tumor (WT), the most common kidney tumor among children, is characterized by a triphasic morphology consisting of blastemal, epithelial, and stromal components. Adult WT is a rare malignancy displaying similar histological features. We here present the first published high-resolution genomic analysis of a mixed-type adult WT. This revealed a more pronounced genetic complexity than usually observed in children with mixed-type WT. The majority of chromosomes displayed uniparental disomies, and microdeletions were present in genes with known importance for tumor formation (LRP1B, FHIT, and WWOX) or organogenesis (NEGR1 and ZFPM2), abnormalities not previously reported for pediatric WT. Our results indicate that adult WT is a biological... (More)
Wilms' tumor (WT), the most common kidney tumor among children, is characterized by a triphasic morphology consisting of blastemal, epithelial, and stromal components. Adult WT is a rare malignancy displaying similar histological features. We here present the first published high-resolution genomic analysis of a mixed-type adult WT. This revealed a more pronounced genetic complexity than usually observed in children with mixed-type WT. The majority of chromosomes displayed uniparental disomies, and microdeletions were present in genes with known importance for tumor formation (LRP1B, FHIT, and WWOX) or organogenesis (NEGR1 and ZFPM2), abnormalities not previously reported for pediatric WT. Our results indicate that adult WT is a biological entity distinct from the corresponding pediatric tumor type. (Less)
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type
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publication status
published
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in
Virchows Archiv: an international journal of pathology
volume
459
pages
547 - 553
publisher
Springer
external identifiers
  • wos:000296468800010
  • pmid:21947875
  • scopus:80855143997
ISSN
1432-2307
DOI
10.1007/s00428-011-1148-0
language
English
LU publication?
yes
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The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Division of Clinical Genetics (013022003), Pediatrics/Urology/Gynecology/Endocrinology (013240400)
id
944563a8-c881-4d4d-9eab-292fd1175c61 (old id 2168401)
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http://www.ncbi.nlm.nih.gov/pubmed/21947875?dopt=Abstract
date added to LUP
2016-04-04 09:37:27
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2022-01-29 18:43:24
@article{944563a8-c881-4d4d-9eab-292fd1175c61,
  abstract     = {{Wilms' tumor (WT), the most common kidney tumor among children, is characterized by a triphasic morphology consisting of blastemal, epithelial, and stromal components. Adult WT is a rare malignancy displaying similar histological features. We here present the first published high-resolution genomic analysis of a mixed-type adult WT. This revealed a more pronounced genetic complexity than usually observed in children with mixed-type WT. The majority of chromosomes displayed uniparental disomies, and microdeletions were present in genes with known importance for tumor formation (LRP1B, FHIT, and WWOX) or organogenesis (NEGR1 and ZFPM2), abnormalities not previously reported for pediatric WT. Our results indicate that adult WT is a biological entity distinct from the corresponding pediatric tumor type.}},
  author       = {{Karlsson, Jenny and Holmquist Mengelbier, Linda and Elfving, Peter and Gisselsson Nord, David}},
  issn         = {{1432-2307}},
  language     = {{eng}},
  pages        = {{547--553}},
  publisher    = {{Springer}},
  series       = {{Virchows Archiv:  an international journal of pathology}},
  title        = {{High-resolution genomic profiling of an adult Wilms' tumor: evidence for a pathogenesis distinct from corresponding pediatric tumors.}},
  url          = {{http://dx.doi.org/10.1007/s00428-011-1148-0}},
  doi          = {{10.1007/s00428-011-1148-0}},
  volume       = {{459}},
  year         = {{2011}},
}