Document Detail


Malignant melanoma and Wiedemann-Beckwith syndrome in childhood.
MedLine Citation:
PMID:  21058226     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Patients with Wiedemann-Beckwith syndrome (WBS, MIM 130650), a congenital overgrowth syndrome, have a known increased tumor risk especially for embryonic tumors. WBS belongs to the "imprinting" syndromes caused by overexpression of IGF2 and/or loss of CDKN1C on chromosome 11p15.5. A 13-year-old boy with WBS developed a spitzoid malignant melanoma (Clark level V, Breslow index 4.8 mm) on the right cheek. Genetic analyses of the patient's blood showed hypermethylation at the H19 locus on chromosome 11p. The (epi)genetic changes of the WBS locus might have played a role in the pathogenesis of melanoma development.
Authors:
E Livingstone; A Caliebe; F Egberts; E Proksch; K Buiting; C Schubert; A Claviez; R Siebert; A Hauschild
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-11-05
Journal Detail:
Title:  Klinische Pädiatrie     Volume:  222     ISSN:  1439-3824     ISO Abbreviation:  Klin Padiatr     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-11-08     Completed Date:  2011-03-29     Revised Date:  2011-10-07    
Medline Journal Info:
Nlm Unique ID:  0326144     Medline TA:  Klin Padiatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  388-90     Citation Subset:  IM    
Copyright Information:
© Georg Thieme Verlag KG Stuttgart · New York.
Affiliation:
Department of Dermatology, University Hospital Duisburg-Essen, Essen, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Beckwith-Wiedemann Syndrome / diagnosis*,  genetics
Cheek
Chromosomes, Human, Pair 11 / genetics*
DNA Methylation / genetics*
Facial Neoplasms / diagnosis*,  genetics
Genetic Predisposition to Disease / genetics
Genomic Imprinting / genetics*
Humans
Male
Melanoma / diagnosis*,  genetics
RNA, Untranslated / genetics
Skin Neoplasms / diagnosis*,  genetics
Chemical
Reg. No./Substance:
0/H19 long non-coding RNA; 0/RNA, Untranslated

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