Journal of Neuro-Oncology

, Volume 112, Issue 1, pp 107–114

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

  • Daniel Satgé
  • Charles A. Stiller
  • Stefan Rutkowski
  • André O. von Bueren
  • Brigitte Lacour
  • Danièle Sommelet
  • Motoi Nishi
  • Maura Massimino
  • Maria Luisa Garré
  • Florencia Moreno
  • Henrik Hasle
  • Zsuzsanna Jakab
  • Mark Greenberg
  • Nicolas von der Weid
  • Claudia Kuehni
  • Oscar Zurriaga
  • Maria-Luisa Vicente
  • Rafael Peris-Bonet
  • Martin Benesch
  • Michel Vekemans
  • Sheena G. Sullivan
  • Christian Rickert
Clinical Study

DOI: 10.1007/s11060-012-1041-y

Cite this article as:
Satgé, D., Stiller, C.A., Rutkowski, S. et al. J Neurooncol (2013) 112: 107. doi:10.1007/s11060-012-1041-y

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

Brain tumor Down syndrome Glioma Medulloblastoma Natural protection against cancer Primitive neurectodermal tumor 

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Daniel Satgé
    • 1
  • Charles A. Stiller
    • 2
  • Stefan Rutkowski
    • 3
  • André O. von Bueren
    • 3
  • Brigitte Lacour
    • 4
  • Danièle Sommelet
    • 5
  • Motoi Nishi
    • 6
  • Maura Massimino
    • 7
  • Maria Luisa Garré
    • 8
  • Florencia Moreno
    • 9
  • Henrik Hasle
    • 10
  • Zsuzsanna Jakab
    • 11
  • Mark Greenberg
    • 12
  • Nicolas von der Weid
    • 13
  • Claudia Kuehni
    • 14
  • Oscar Zurriaga
    • 15
  • Maria-Luisa Vicente
    • 16
  • Rafael Peris-Bonet
    • 17
  • Martin Benesch
    • 18
  • Michel Vekemans
    • 19
  • Sheena G. Sullivan
    • 20
  • Christian Rickert
    • 21
  1. 1.Epidemiology and Biostatistics DepartmentEA 2415, University Institute for Clinical Research IURC, Montpellier 1 UniversityMontpellierFrance
  2. 2.Department of PediatricsChildhood Cancer Research Group, University of OxfordOxfordUK
  3. 3.Department of Pediatric Hematology and OncologyUniversity Medical CenterHamburg-EppendorfGermany
  4. 4.Registre National des Tumeurs Solides de l’Enfant, University HospitalNancyFrance
  5. 5.Registre National des Tumeurs Solides de l’Enfant, Faculty of MedicineNancyFrance
  6. 6.Department of Fundamental Health SciencesHealth Sciences University of HokkaidoTobetsuJapan
  7. 7.Pediatric Unit, Fondazione IRCCS Istituto Nazionale TumoriMilanItaly
  8. 8.Neuro Oncology Unit, Giannina Gaslini Children’s Research Hospital (IRCCS)GenoaItaly
  9. 9.Registro Oncopediatrico Hospitalario ArgentinoBuenos AiresArgentina
  10. 10.Department of PediatricAarhus University Hospital SkejbyAarhusDenmark
  11. 11.2nd Department of Pediatrics, Hungarian Pediatric Cancer Registry, Semmelweis UniversityBudapestHungary
  12. 12.Pediatric Oncology Group of Ontario, University of TorontoTorontoCanada
  13. 13.Department of PediatricsUniversity HospitalLausanneSwitzerland
  14. 14.Swiss Childhood Cancer Registry, University of BernBernSwitzerland
  15. 15.Area de Epidemiologia, Direccion General de Salud PublicaValenciaSpain
  16. 16.C. Valencia Childhood Cancer Registry, Epidemiology DepartmentValenciaSpain
  17. 17.National Childhood Cancer Registry (RNTI-SEHOP), University of ValenciaValenciaSpain
  18. 18.Division of Pediatric Hematology and Oncology, Department of Pediatric and Adolescent MedicineMedical University of GrazGrazAustria
  19. 19.Service Histology Embryology Cytogenetics, Hopital Necker Enfants Malades, INSERM U781, and Université DescartesParisFrance
  20. 20.School of Exercise, Biomedical and Health Science, Edith Cowan UniversityPerthAustralia
  21. 21.Department of PathologyVivantes ClinicsBerlinGermany

Personalised recommendations