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Retrospective study of diffuse intrinsic pontine glioma in the Belgian population: a 25 year experience

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Abstract

Introduction

Diffuse intrinsic pontine glioma is a rare disease with a high mortality. Our primary aim was to determine the incidence of this disease in Belgium. Secondly, we wanted to compare the treatment approach of Belgian pediatric oncology centres, to investigate possibilities for improvement.

Methods

We retrospectively collected and analysed data on DIPG-patients diagnosed between 1994 and 2018 and recorded in the Belgian Cancer Registry. We included patients ≤ 18 years who were followed in one of the eight Belgian pediatric oncology centres.

Results

We included 100 patients. Files were complete in 87 patients. We observed an increase in diagnoses with an incidence of 3.1 per 1,000,000 persons (aged 0–≤ 18) per year over the last 5 years compared to an overall incidence of 1.8. Biopsy was performed at diagnosis in 51.7% of patients. In one fifth this was study-related. Mutation analysis was known in eight patients, of which six showed the H3 K27M-mutation. 58.8% of patients received chemotherapy, without a significant survival benefit. 12.6% of patients were included in a clinical trial. Biopsy rate and the use of chemotherapy differed widely between centres. Mean OS and PFS were 10.49 and 4.87 months respectively. We observed an improved survival over time.

Conclusions

Over the past 25 years, we observed an increase of new DIPG-diagnoses. Outcome in our cohort is comparable with literature findings. We demonstrate an important heterogeneity in treatment approach between different centres and limited inclusion in clinical trials. Therefore, collaboration between centres and inclusion of patients in clinical trials is much needed.

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Data availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

We thank Morgan Collin, Caroline De Schepper, Annouschka Laenen, Caroline Martin, An Michiels, Wiert Robberechts, Catherine Sondag, Sam Van Gerwen, Merry Van Puyvelde, Michel Vanden Eynden and Nancy Van Damme for their contribution to this study.

Funding

No funds, grants, or other support was received.

Author information

Authors and Affiliations

  1. Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium

    Dries Ruttens

  2. Department of Pediatric Hematology-Oncology, University Hospitals Leuven, Leuven, Belgium

    Julie Messiaen & Sandra Jacobs

  3. Department of Pediatric Hematology-Oncology, Queen Fabiola Children’s University Hospital, Brussels, Belgium

    Alina Ferster

  4. Department of Pediatrics, Division of Hematology-Oncology, CHU of Liège, Liège, Belgium

    Caroline Piette

  5. Department of Pediatric Hematology-Oncology, CHC MontLégia, Liège, Belgium

    Stefan Schifflers

  6. Department of Pediatric Hematology-Oncology, Saint-Luc University Hospital, Brussels, Belgium

    An Van Damme

  7. Department of Pediatric Hematology-Oncology, University Hospitals Brussel, Brussels, Belgium

    Jutte van der Werff ten Bosch

  8. Department of Pediatric Hematology-Oncology, University Hospital of Antwerp, Edegem, Belgium

    Joris Verlooy

  9. Department of Pediatric Hematology-Oncology, University Hospital Ghent, Ghent, Belgium

    Leen Willems

Authors
  1. Dries Ruttens
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  4. Caroline Piette
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  10. Sandra Jacobs
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Contributions

All authors contributed to the study conception and design. Data collection was performed by DR, data analysis was performed by DR and JM. The first draft of the manuscript was written by DR and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Dries Ruttens.

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Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committees of the different centres.

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Ruttens, D., Messiaen, J., Ferster, A. et al. Retrospective study of diffuse intrinsic pontine glioma in the Belgian population: a 25 year experience. J Neurooncol 153, 293–301 (2021). https://doi.org/10.1007/s11060-021-03766-y

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