Researchers from the Institute of Cancer Research (ICR) have identified a new treatment pathway for a rare set of childhood brain tumours known as gliomatosis cerebri.
Published in the journal Neuro-Oncology and funded by the Rudy A Menon Foundation and the Ollie Young Foundation, the decade-long study could lead to more effective treatments to treat this set of conditions.
Affecting approximately 100 people every year in the US, gliomatosis cerebri is a highly aggressive form of glioma that presents as a spider’s web of cancer threads that spread deep into the brain.
No chemotherapy has proven to be effective when treating this form of brain cancer, which is almost impossible to remove with surgery and is difficult to treat with radiotherapy.
Carried out by the High-Grade Glioma Working Group on behalf of the International Society for Paediatric Oncology Europe, researchers collected brain tissue samples to carry out the study involving 104 children and adolescents living with the disease.
The team used tumour profiling to understand what molecules are driving cancer and was able to identify a molecular profile for gliomatosis cerebri.
The results revealed two subtypes known as pedHGG_RTK2A/B and pedHGG_A/B, which were both associated with paediatric gliomatosis cerebri.
In addition, researchers revealed that mutations in the epidermal growth factor receptor protein and the BCOR gene, along with rearrangements of chromosome 6, were the most common genetic features.
Findings from the study could help researchers identify potential drug targets for this condition.
Chris Jones, professor of childhood brain tumour biology, ICR, commented: “We demonstrated that gliomatosis cerebri tumours behave very aggressively and differ from other types of gliomas found in children.
“Contrary to previous research, the… study has shown that there are distinct molecular and clinical features of gliomatosis cerebri and this provides evidence that it ought to be re-considered as a separate entity.”
Co-founder of the Rudy A Menon Foundation, Vidhu Menon, said that this “research has led to significant progress” and “will become the cornerstone for further understanding and finding effective treatments for this disease globally”.