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



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.


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.


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.


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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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.


No funds, grants, or other support was received.

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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.

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Correspondence to Dries Ruttens.

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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 (2021).

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  • Diffuse intrinsic pontine glioma
  • Brainstem glioma
  • Diffuse midline glioma
  • Biopsy
  • Radiotherapy
  • Chemotherapy