A lack of neuroblastoma in Down syndrome: a study from 11 European countries
Details
Serval ID
serval:BIB_A2697A55BE3F
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
A lack of neuroblastoma in Down syndrome: a study from 11 European countries
Journal
Cancer Research
ISSN
0008-5472 (Print)
Publication state
Published
Issued date
02/1998
Volume
58
Number
3
Pages
448-52
Notes
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Feb 1
Research Support, Non-U.S. Gov't --- Old month value: Feb 1
Abstract
An epidemiological investigation in 11 European countries comprising a total childhood population of 54.1 million children and using 8 separate data sources was conducted to evaluate the occurrence of neuroblastoma in Down syndrome (DS). No cases of DS were detected among 6724 infants and children with neuroblastoma, although more than five were expected. This highly significant result (P = 0.0045 according to the Poisson test) is consistent with data in the literature, which contains only two poorly detailed cases in epidemiological studies and one ganglioneuroma in a DS mosaic patient. Like other tumors, such as leukemias, testicular germ cell tumors and lymphomas are in excess in DS patients; the lack of neuroblastomas does not reflect a general decreased incidence of cancer but rather a specific underrepresentation of this precise tumor. S-100 b protein, the gene for which maps to the long arm of chromosome 21, (a) is overproduced in DS patients, (b) produces growth inhibition and differentiation of neural cells in vitro, (c) is abundant in good-prognosis neuroblastomas, and (d) has been shown to induce growth inhibition and differentiation and cell death in several human and murine neuroblastoma cell lines and could be responsible for this variation. Additional epidemiological and experimental studies are warranted to confirm our interpretation of these data.
Keywords
Adolescent
Child
Child, Preschool
Chromosomes, Human, Pair 21/genetics
Comorbidity
Down Syndrome/*epidemiology/genetics
Europe/epidemiology
Female
Humans
Immunity, Natural
Incidence
Infant
Infant, Newborn
Male
Neuroblastoma/*epidemiology/genetics
S100 Proteins/genetics/physiology
Pubmed
Web of science
Create date
25/01/2008 11:35
Last modification date
20/08/2019 16:08