Dries Ruttens1, Julie Messiaen2, Alina Ferster3, Caroline Piette4, Stefan Schifflers5, An Van Damme6, Jutte van der Werff Ten Bosch7, Joris Verlooy8, Leen Willems9, Sandra Jacobs2. 1. Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium. dries.ruttens@student.kuleuven.be. 2. Department of Pediatric Hematology-Oncology, University Hospitals Leuven, Leuven, Belgium. 3. Department of Pediatric Hematology-Oncology, Queen Fabiola Children's University Hospital, Brussels, Belgium. 4. Department of Pediatrics, Division of Hematology-Oncology, CHU of Liège, Liège, Belgium. 5. Department of Pediatric Hematology-Oncology, CHC MontLégia, Liège, Belgium. 6. Department of Pediatric Hematology-Oncology, Saint-Luc University Hospital, Brussels, Belgium. 7. Department of Pediatric Hematology-Oncology, University Hospitals Brussel, Brussels, Belgium. 8. Department of Pediatric Hematology-Oncology, University Hospital of Antwerp, Edegem, Belgium. 9. Department of Pediatric Hematology-Oncology, University Hospital Ghent, Ghent, Belgium.
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.
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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