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Embryonal tumors in Canadian children less than 36 months of age: results from the Canadian Pediatric Brain Tumor Consortium (CPBTC)

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Abstract

Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005. Embryonal tumors accounted for 26.9% of all CNS tumors. Medulloblastomas were the highest proportion of the embryonal tumors at 61.5%. Atypical teratoid/rhabdoid tumors (AT/RT) had the second highest proportion of embryonal tumors at 18%. The proportion of primitive neuroectodermal tumors (PNET) was 16%, with 2.6 and 1.9% for congenital medulloepithelioma and ependymoblastoma tumors, respectively. AT/RT and PNET were more common in younger age groups. Medulloblastoma became more prevalent with increasing age, with its highest prevalence in the 25 to 36 month age group. Survival rates for our Canadian population at 18 and 24 months were 0.74 and 0.68 for medulloblastoma, 0.64 and 0.60 for PNET, and 0.36 and 0.29 for AT/RT, respectively. Overall, our data are comparable with published international rates for embryonal tumors. These incidence and outcome figures can guide future research into these rare tumors.

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Funding

This study was not funded.

Author information

Authors and Affiliations

  1. Division of Pediatric Hematology/Oncology, Children’s Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada

    E. Story & D. L. Johnston

  2. Division of Pediatric Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada

    U. Bartels & E. Bouffet

  3. Division of Pediatric Hematology/Oncology, Centre Hospitalier Universitaire Sainte-Justine, Montreal, QC, Canada

    A. S. Carret

  4. Division of Pediatric Hematology/Oncology, IWK Health Centre, Halifax, NS, Canada

    B. Crooks

  5. Division of Pediatric Hematology/Oncology, Stollery Children’s Hospital, Edmonton, AB, Canada

    D. D. Eisenstat & B. Wilson

  6. Division of Pediatric Hematology/Oncology, BC Children’s Hospital, Vancouver, BC, Canada

    C. Fryer

  7. Division of Oncology, Alberta Children’s Hospital, Calgary, AB, Canada

    L. Lafay-Cousin

  8. Division of Pediatric Hematology/Oncology, Centre Hospitalier Universitaire de Quebec, Quebec City, QC, Canada

    V. Larouche

  9. Division of Pediatric Hematology/Oncology, Children’s Hospital of Western Ontario, London, ON, Canada

    S. Zelcer

  10. Division of Pediatric Hematology/Oncology, Kingston General Hospital, Kingston, ON, Canada

    M. Silva

  11. Division of Pediatric Hematology/Oncology, Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, QC, Canada

    J. Brossard

  12. Division of Pediatric Neurology, Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada

    D. L. Keene

Authors
  1. E. Story
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  2. D. L. Johnston
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  3. U. Bartels
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  4. A. S. Carret
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  7. C. Fryer
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  8. L. Lafay-Cousin
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  9. V. Larouche
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  10. B. Wilson
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  11. S. Zelcer
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  12. M. Silva
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  13. J. Brossard
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  14. E. Bouffet
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  15. D. L. Keene
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Corresponding author

Correspondence to E. Story.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This study does not contain any studies with animals performed by an of the authors.

Informed consent

This study was a retrospective chart review. For this type of study formal consent is not required.

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Story, E., Johnston, D.L., Bartels, U. et al. Embryonal tumors in Canadian children less than 36 months of age: results from the Canadian Pediatric Brain Tumor Consortium (CPBTC). J Neurooncol 133, 581–587 (2017). https://doi.org/10.1007/s11060-017-2468-y

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  • DOI: https://doi.org/10.1007/s11060-017-2468-y

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