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A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries

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A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries. / Satgé, Daniel; Stiller, Charles A; Rutkowski, Stefan; von Bueren, André O; Lacour, Brigitte; Sommelet, Danièle; Nishi, Motoi; Massimino, Maura; Garré, Maria Luisa; Moreno, Florencia; Hasle, Henrik; Jakab, Zsuzsanna; Greenberg, Mark; von der Weid, Nicolas; Kuehni, Claudia; Zurriaga, Oscar; Vicente, Maria-Luisa; Peris-Bonet, Rafael; Benesch, Martin; Vekemans, Michel; Sullivan, Sheena G; Rickert, Christian.

in: J NEURO-ONCOL, Jahrgang 112, Nr. 1, 01.03.2013, S. 107-14.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Satgé, D, Stiller, CA, Rutkowski, S, von Bueren, AO, Lacour, B, Sommelet, D, Nishi, M, Massimino, M, Garré, ML, Moreno, F, Hasle, H, Jakab, Z, Greenberg, M, von der Weid, N, Kuehni, C, Zurriaga, O, Vicente, M-L, Peris-Bonet, R, Benesch, M, Vekemans, M, Sullivan, SG & Rickert, C 2013, 'A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries', J NEURO-ONCOL, Jg. 112, Nr. 1, S. 107-14. https://doi.org/10.1007/s11060-012-1041-y

APA

Satgé, D., Stiller, C. A., Rutkowski, S., von Bueren, A. O., Lacour, B., Sommelet, D., Nishi, M., Massimino, M., Garré, M. L., Moreno, F., Hasle, H., Jakab, Z., Greenberg, M., von der Weid, N., Kuehni, C., Zurriaga, O., Vicente, M-L., Peris-Bonet, R., Benesch, M., ... Rickert, C. (2013). A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries. J NEURO-ONCOL, 112(1), 107-14. https://doi.org/10.1007/s11060-012-1041-y

Vancouver

Satgé D, Stiller CA, Rutkowski S, von Bueren AO, Lacour B, Sommelet D et al. A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries. J NEURO-ONCOL. 2013 Mär 1;112(1):107-14. https://doi.org/10.1007/s11060-012-1041-y

Bibtex

@article{38cd756007b840809a5eb56bbdfe6d61,
title = "A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries",
abstract = "Persons with Down syndrome (DS) uniquely have an increased frequency of leukemias but a decreased total frequency of solid tumors. The distribution and frequency of specific types of brain tumors have never been studied in DS. We evaluated the frequency of primary neural cell embryonal tumors and gliomas in a large international data set. The observed number of children with DS having a medulloblastoma, central nervous system primitive neuroectodermal tumor (CNS-PNET) or glial tumor was compared to the expected number. Data were collected from cancer registries or brain tumor registries in 13 countries of Europe, America, Asia and Oceania. The number of DS children with each category of tumor was treated as a Poisson variable with mean equal to 0.000884 times the total number of registrations in that category. Among 8,043 neural cell embryonal tumors (6,882 medulloblastomas and 1,161 CNS-PNETs), only one patient with medulloblastoma had DS, while 7.11 children in total and 6.08 with medulloblastoma were expected to have DS. (p 0.016 and 0.0066 respectively). Among 13,797 children with glioma, 10 had DS, whereas 12.2 were expected. Children with DS appear to be specifically protected against primary neural cell embryonal tumors of the CNS, whereas gliomas occur at the same frequency as in the general population. A similar protection against neuroblastoma, the principal extracranial neural cell embryonal tumor, has been observed in children with DS. Additional genetic material on the supernumerary chromosome 21 may protect against embryonal neural cell tumor development.",
keywords = "Cerebellar Neoplasms, Child, Child, Preschool, Down Syndrome, Female, Humans, Male, Medulloblastoma, World Health",
author = "Daniel Satg{\'e} and Stiller, {Charles A} and Stefan Rutkowski and {von Bueren}, {Andr{\'e} O} and Brigitte Lacour and Dani{\`e}le Sommelet and Motoi Nishi and Maura Massimino and Garr{\'e}, {Maria Luisa} and Florencia Moreno and Henrik Hasle and Zsuzsanna Jakab and Mark Greenberg and {von der Weid}, Nicolas and Claudia Kuehni and Oscar Zurriaga and Maria-Luisa Vicente and Rafael Peris-Bonet and Martin Benesch and Michel Vekemans and Sullivan, {Sheena G} and Christian Rickert",
year = "2013",
month = mar,
day = "1",
doi = "10.1007/s11060-012-1041-y",
language = "English",
volume = "112",
pages = "107--14",
journal = "J NEURO-ONCOL",
issn = "0167-594X",
publisher = "Kluwer Academic Publishers",
number = "1",

}

RIS

TY - JOUR

T1 - A very rare cancer in Down Syndrome: medulloblastoma. Epidemiological data from 13 countries

AU - Satgé, Daniel

AU - Stiller, Charles A

AU - Rutkowski, Stefan

AU - von Bueren, André O

AU - Lacour, Brigitte

AU - Sommelet, Danièle

AU - Nishi, Motoi

AU - Massimino, Maura

AU - Garré, Maria Luisa

AU - Moreno, Florencia

AU - Hasle, Henrik

AU - Jakab, Zsuzsanna

AU - Greenberg, Mark

AU - von der Weid, Nicolas

AU - Kuehni, Claudia

AU - Zurriaga, Oscar

AU - Vicente, Maria-Luisa

AU - Peris-Bonet, Rafael

AU - Benesch, Martin

AU - Vekemans, Michel

AU - Sullivan, Sheena G

AU - Rickert, Christian

PY - 2013/3/1

Y1 - 2013/3/1

N2 - Persons with Down syndrome (DS) uniquely have an increased frequency of leukemias but a decreased total frequency of solid tumors. The distribution and frequency of specific types of brain tumors have never been studied in DS. We evaluated the frequency of primary neural cell embryonal tumors and gliomas in a large international data set. The observed number of children with DS having a medulloblastoma, central nervous system primitive neuroectodermal tumor (CNS-PNET) or glial tumor was compared to the expected number. Data were collected from cancer registries or brain tumor registries in 13 countries of Europe, America, Asia and Oceania. The number of DS children with each category of tumor was treated as a Poisson variable with mean equal to 0.000884 times the total number of registrations in that category. Among 8,043 neural cell embryonal tumors (6,882 medulloblastomas and 1,161 CNS-PNETs), only one patient with medulloblastoma had DS, while 7.11 children in total and 6.08 with medulloblastoma were expected to have DS. (p 0.016 and 0.0066 respectively). Among 13,797 children with glioma, 10 had DS, whereas 12.2 were expected. Children with DS appear to be specifically protected against primary neural cell embryonal tumors of the CNS, whereas gliomas occur at the same frequency as in the general population. A similar protection against neuroblastoma, the principal extracranial neural cell embryonal tumor, has been observed in children with DS. Additional genetic material on the supernumerary chromosome 21 may protect against embryonal neural cell tumor development.

AB - Persons with Down syndrome (DS) uniquely have an increased frequency of leukemias but a decreased total frequency of solid tumors. The distribution and frequency of specific types of brain tumors have never been studied in DS. We evaluated the frequency of primary neural cell embryonal tumors and gliomas in a large international data set. The observed number of children with DS having a medulloblastoma, central nervous system primitive neuroectodermal tumor (CNS-PNET) or glial tumor was compared to the expected number. Data were collected from cancer registries or brain tumor registries in 13 countries of Europe, America, Asia and Oceania. The number of DS children with each category of tumor was treated as a Poisson variable with mean equal to 0.000884 times the total number of registrations in that category. Among 8,043 neural cell embryonal tumors (6,882 medulloblastomas and 1,161 CNS-PNETs), only one patient with medulloblastoma had DS, while 7.11 children in total and 6.08 with medulloblastoma were expected to have DS. (p 0.016 and 0.0066 respectively). Among 13,797 children with glioma, 10 had DS, whereas 12.2 were expected. Children with DS appear to be specifically protected against primary neural cell embryonal tumors of the CNS, whereas gliomas occur at the same frequency as in the general population. A similar protection against neuroblastoma, the principal extracranial neural cell embryonal tumor, has been observed in children with DS. Additional genetic material on the supernumerary chromosome 21 may protect against embryonal neural cell tumor development.

KW - Cerebellar Neoplasms

KW - Child

KW - Child, Preschool

KW - Down Syndrome

KW - Female

KW - Humans

KW - Male

KW - Medulloblastoma

KW - World Health

U2 - 10.1007/s11060-012-1041-y

DO - 10.1007/s11060-012-1041-y

M3 - SCORING: Journal article

C2 - 23307327

VL - 112

SP - 107

EP - 114

JO - J NEURO-ONCOL

JF - J NEURO-ONCOL

SN - 0167-594X

IS - 1

ER -