New insights on the genetic drivers behind a rare type of fatal pediatric brain cancer may lead to the development of new patient-targeted treatments, a new study suggests.

Each year about 30 children in Canada are diagnosed with diffuse intrinsic pontine glioma (DIPG), a pediatric cancer for which there is no effective treatment.

Because DIPG tumours occur in the middle of the brainstem specifically in the "pons" region that controls vital functions such as breathing, heart regulation and movement doctors are not able to surgically remove them.

As well, biopsies are rarely performed on the tumours, meaning researchers have faced challenges investigating the genetic landscape of DIPG.

But in a new study from Toronto's Hospital for Sick Children, researchers have uncovered the genetic drivers of DIPG.

The study, published online Sunday in the journal Nature Genetics, found that DIPG is comprised of three molecularly distinct subgroups: "MYCN," "silent" and "H3-K27M."

"Although previously considered to be one disease, DIPG represents three distinct subgroups with different methylation, expression, copy number alteration (CAN) and mutational profiles," the authors write.

The results from the study show that DIPG tumours are distinct from adult brain cancer, the study says.

Dr. Cynthia Hawkins, a neuropathologist and the study's principal investigator, said the discovery will have a significant impact on the development of DIPG treatment options.

"This work gives us the opportunity to make some real progress for these patients and their families," she said in a statement.

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New study sheds light on genetic drivers of fatal pediatric cancer

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