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.
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Acknowledgments
A grant from the Fondation Jérôme Lejeune supports the study on cancer in Down syndrome (Daniel Satgé). Authors greatly acknowledge the data provided by the Australian Paediatric Cancer Registry. We thank the Deutsche Kinderkrebsstiftung for its support to the paediatric brain tumour trial office HIT. The Childhood Cancer Research Group receives funding from the Department of Health, the Scottish Government, the National Cancer Intelligence Network and Children with Cancer UK. The views expressed here are those of the authors and not necessarily those of any of these organisations. The National childhood cancer registry of Spain is in the RTICC (RD/06/0020/0033) and the CIBERESP, and it receives funding from the Scientific Foundation of the AECC and the Villavecchia Foundation. We also thank the notifying centres of the province of Barcelona for the data provided. We are grateful to Christiane Satgé who prepared the manuscript.
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Satgé, D., Stiller, C.A., Rutkowski, S. et al. A very rare cancer in Down syndrome: medulloblastoma. Epidemiological data from 13 countries. J Neurooncol 112, 107–114 (2013). https://doi.org/10.1007/s11060-012-1041-y
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DOI: https://doi.org/10.1007/s11060-012-1041-y