Ependymoma is a childhood brain cancer, a rare disease that has a survival rate of about 50%. Not much is known about its biology, but scientists at Nottingham University in the UK have pinpointed three genes involved in its development.
Ependymoma - A rare disease
Childhood ependymoma is a disease in which a tumour forms from the glial cells, the supporting cells of the central nervous system. About 10% of childhood brain tumours are ependymomas and the current five-year survival rate stands at about 50%.
The new research, which was published in the British Journal of Cancer should help to provide a better understanding of the basic genetics and physiological mechanisms of ependymomas as well as leading to the creation of drugs that are better at targeting tumours and producing fewer side effects.
Lead author Professor Richard Grundy from Nottingham University is hopeful about the value of his team's findings. "We hope our findings will lead to a more detailed understanding of ependymoma. This is crucial if we are to ensure each child receives the most appropriate treatment for their disease and that we reduce the number of children in which their cancer recurs."
Genes for brain cancer
The scientists studied the basic gene activity of 74 samples of ependymomas on chromosome 1. The team had previously linked the chromosome with poor survival rates for ependymomas.
The 3 genes they discovered were;
SI00A4 - it had a strong association with tumours in young children
SI00A6 - a marker for a brain tumour
CHI3L1 - this was common in cancers with a larger degree of cell death.
Understanding basic genetics
One of the problems with chemotherapy as treatment is that it's not always perfect at targeting the specific cancer cells without causing collateral damage to nearby cells. Deeper knowledge of the genetic mechanisms involved in the cancer will help with more precision targeting. Surgery and radiation are also treatment options. And as with any cancer, in fact with any genetic disorder, 'know thine enemy' is key. A fuller understanding of what takes place at the genetic level can lead to ways of exploiting and subverting pathways that lead to tumour development, and so developing new brain cancer treatments and increasing the rates of patient survival.
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