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

• DOI: 10.1007/s11060-021-03766-y
• Publication Date: 2021-05-03T20:07:16Z
• Authors: Dries Ruttens; Julie Messiaen; Alina Ferster; Caroline Piette; Stefan Schifflers; An Van Damme; Jutte van der Wer...; Joris Verlooy; Leen Willems; Sandra Jacobs
• Keywords: Diffuse intrinsic pontine glioma; Biopsy; chemotherapy; brainstem; 03 medical and health sciences; 0302 clinical medicine; Belgium; glioma; Neurologie; Chemotherapy; Humans; biopsy; Child; Brainstem glioma; radiotherapy; Retrospective Studies; Radiotherapy; brainstem glioma; Diffuse Intrinsic Pontine Glioma; diffuse intrinsic pontine glioma; Diffuse midline glioma; Glioma; 3. Good health; Cancérologie; diffuse midline glioma; Human medicine; RADIOTHERAPY

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 over a period of 25 years with a median age at diagnosis of 7 years. 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. Forty-five patients (51.7%) were biopsied at diagnosis. In ten (22%), this was study-related. H3 K27M-mutation was present in 75% of biopsied patients. Fifty-one patients (59.3%) received chemotherapy, without a significant survival benefit. Eleven patients (21.2%) 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.