Diffuse intrinsic brainstem gliomas constitute 15-20% of all CNS tumours in children, and are the main cause of death in children with brain tumours. Many clinical trials have been done over the past three decades, but survival has remained static. More than 90% of children die within 2 years of diagnosis, and conventional fractionated radiation remains the standard treatment. However, median survival differs substantially between clinical trials, suggesting a survival benefit with some strategies. We appraised the consistency between protocols in terms of eligibility criteria, definition and assessment of response and progression, statistical design, and endpoints. Study designs varied substantially, which could explain the differences in outcome, and no treatment has shown a benefit over conventional radiotherapy. However, consistency between protocols (eg, eligibility criteria and outcome measures) is important to measure the progress in management of diffuse pontine gliomas.
ContributorsHargrave, Darren and Ute Bartels, Eric Bouffet
Disease Category: Cancer, Child health, Neurology
Disease Name: Gliomas
Age Range: 0 - 18
Sex: Either
Nature of Intervention: Chemotherapy , Radiotherapy
- Clinical experts
- Overview of literature
- Literature review
Data for this review were identified by searches of the PubMed database from 1975 to the present. Search terms included: “brainstem” or “brain stem”; “glioma” or “gliomas”; “tumor” or “tumors”; “diffuse”; “pontine”; “radiotherapy”; and “chemotherapy”. Reports were also identified from references from relevant articles. The search was limited to the paediatric and young adult populations (1–18 years) reported in English. Only prospective clinical trials with newly diagnosed patients were selected. Letters to editor, case reports, repeat publications, commentaries, and meeting abstracts were excluded. Studies that included a mixed group of patients with brainstem and non-brainstem glioma or paediatric and adult patients were included; however, such studies had to include a specific subanalysis of the population with paediatric brainstem glioma. To improve the quality of the information found by the search, a copy of the trial protocols was obtained, either through the corresponding oncology group website or by contact with the first author of the article.