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

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 patien... Mehr ...

Verfasser: Ruttens, Dries
Messiaen, Julie
Ferster, Alina
Piette, Caroline
Schifflers, Stefan
Van Damme, An
van der Werff ten Bosch, Jutte
Verlooy, Joris
Willems, Leen
Jacobs, Sandra
Dokumenttyp: Artikel
Erscheinungsdatum: 2021
Schlagwörter: Human medicine
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-28877290
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://hdl.handle.net/10067/1782050151162165141

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.