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

Abstract

IntroductionDiffuse 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. MethodsWe 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. ResultsWe 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-ve 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 signi cant survival bene t. 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. ConclusionsOver the past 25 years, we observed an increase of new DIPG-diagnoses. Outcome in our cohort is comparable with literature ndings. 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.

High Level Immunoglobulin Gene Expression and Reduced Intra-tumoral Bacteria Associated With the Transition from Initial to Progressive Diffuse Intrinsic Pontine Glioma

“…Representing about 15% of childhood brain tumors (1), DIPG has a median age of diagnosis in children of about 6 years, with 90% loss of life within two years of diagnosis (2,3). Current standard of care consists of radiation therapy, given that the tumor location is generally not amenable to resection and that chemotherapy has not consistently led to improved outcomes (3,4). However, DIPG-related clinical trials have been increasing in number in the past decade (5), offering some hope for improved patient outcomes with new pharmacological treatments.…”

Incidence, survival, and mortality of cancer in children and young adolescents in Belgium and the Netherlands in 2004–2015: A comparative population‐based study

“…For CNS tumors, a high proportion of microscopically confirmed cases (>90%) may indicate incomplete and selective registration because part of the CNS tumors are diagnosed by imaging only 5 . Within the malignant astrocytomas & gliomas group these include, for example, diffuse midline gliomas located in the pons, which have a dismal outcome 26,27 . Not including these aggressive tumors will most likely result in higher survival outcomes.…”

“…5 Within the malignant astrocytomas & gliomas group these include, for example, diffuse midline gliomas located in the pons, which have a dismal outcome. 26,27 Not including these aggressive tumors will most likely result in higher survival outcomes. An in-depth analysis of treatment characteristics of Dutch children with malignant CNS tumors will be the subject of future research to further examine the temporary deterioration in survival between 2000 and 2009.…”

H3 K27M-Altered Diffuse Midline Gliomas: A Review

“…Prognosis DMG H3 K27M-altered carry a poor prognosis for both adults and children, with a median survival around 1 year and a poorer prognosis compared to most other gliomas (►Table 4). 28,29,36,40,53,59,67,86,92 In adults, prognosis does not vary by anatomical site. 10 In children with DMG H3 K27M-mutant, the median survival is 9 to 15 months, 89 except in children with NF1 for whom all DMG have an extremely poor prognosis, independent of the presence or absence of H3 K27M mutation.…”