| High incidence of malformation syndromes in a series of 1,073 children with cancer. | |
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MedLine Citation:
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PMID: 15712196 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Constitutional molecular defects are known to play a role in oncogenesis, as shown by the increased incidence of embryonic cancers in children with Beckwith-Wiedemann syndrome (BWS) or of leukemia in children with Down syndrome. To establish the incidence and spectrum of malformation syndromes associated with childhood cancer we performed a clinical morphological examination on a series of 1,073 children with cancer. We diagnosed a syndrome in 42 patients (3.9%) and suspected the presence of a syndrome in another 35 patients (3.3%), for a total of 7.2%. This incidence of patients with a proven or suspected syndrome is high, and points to a possible association. We describe new syndrome-tumor associations in several entities: cleidocranial dysostosis (Wilms tumor), Bardet-Biedl syndrome (BBS) (acute lymphoblastic leukemia), Kabuki syndrome (neuroblastoma), LEOPARD syndrome (neuroblastoma), Poland anomaly (osteosarcoma; Hodgkin disease), and blepharophimosis epicanthus inversus syndrome (Burkitt lymphoma). Twenty of the 42 syndrome diagnoses were not recognized in the patients prior to this study, indicating that these diagnoses are commonly missed. We propose that all children with a malignancy should be examined by a clinical geneticist or a pediatrician skilled in clinical morphology to determine if the patients have a malformation syndrome. |
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Authors:
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Johannes Hans M Merks; Huib N Caron; Raoul C M Hennekam |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: American journal of medical genetics. Part A Volume: 134A ISSN: 1552-4825 ISO Abbreviation: Am. J. Med. Genet. A Publication Date: 2005 Apr |
Date Detail:
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Created Date: 2005-03-24 Completed Date: 2005-06-27 Revised Date: 2008-05-21 |
Medline Journal Info:
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Nlm Unique ID: 101235741 Medline TA: Am J Med Genet A Country: United States |
Other Details:
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Languages: eng Pagination: 132-43 Citation Subset: IM |
Copyright Information:
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(c) 2005 Wiley-Liss, Inc. |
Affiliation:
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Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands. j.h.merks@amc.uva.nl |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
/
diagnosis,
epidemiology*,
genetics Adolescent Adult Child Child, Preschool Cohort Studies Comorbidity DNA Methylation Female Genetic Predisposition to Disease / genetics Humans Incidence Infant Infant, Newborn Male Middle Aged Mutation Neoplasms / epidemiology* Netherlands / epidemiology Syndrome |
| Comments/Corrections | |
Comment In:
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Am J Med Genet A. 2006 Apr 15;140(8):932
[PMID:
16532461
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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