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A new study led by researchers at the Ï㽶ÊÓƵ Health Centre (MUHC) has significantly advanced the understanding of pediatric glioblastoma (pGBM) – the most deadly form of brain tumour. The study, published in the April 1 edition of the Journal of Clinical Oncology, reveals that there are at least two subsets of pGBM, which are activated through different genetic pathways. In addition, their findings confirm that pGBM and the adult form (aGBM) are genetically distinct. These discoveries may lead to improved and disease-specific treatment that will halt tumour growth.

Glioblastoma multiforme, or GBM, is a particularly aggressive type of brain tumour that strikes apparently healthy people, from adults to children, without warning. "Adult GBM (aGBM) kills the vast majority of patients within two years of diagnosis," says Dr. Nada Jabado, a researcher at the Montreal Children's Hospital of the MUHC, an Assistant Professor of Pediatrics at Ï㽶ÊÓƵ, and senior author of the new study. "With around a 20 percent survival rate, pediatric GBM (pGBM) is only slightly less lethal than aGBM.

"Overall, we know very little about pGBM," she notes. "For many years pGBM and aGBM were considered to be the same disease, but we started to notice differences between the two in terms of survival, response to treatment and genetic abnormalities. Our findings confirm that pGBM and aGBM are triggered by different molecular events."

Genetic testing conducted by the researchers, who include Dr. Andre Nantel from the Biotechnology Research Institute (Canadian Research Council), also revealed that there are at least two different forms of pGBM. "Both subsets of pGBM show an abnormal production of a protein that may drive the growth of this tumour," says Dr. Jabado. "We hope this study will provide scientists with better treatment targets for this devastating disease."

Alterations in our genes are responsible for an estimated 5,000 hereditary diseases and influence the development of thousands of other diseases. Once the genes involved in a particular disease are discovered, researchers become better able to precisely diagnose disease, predict its course, and create more effective treatments with fewer side effects. Medical genetics can even be used to assess patients' risk of developing certain diseases, allowing them to take preventive medicines and make lifestyle changes, like diet and environment, which may help prevent development. Medical genetics research is advancing at an incredible rate.

Over the past year MUHC scientists have identified genes responsible for breast cancer, colon cancer, lung cancer, tuberculosis and migraines.

This research was supported by funds from the Canadian Institutes of Health Research, the Penny Cole Foundation, an NRC Genome Health Initiative grant, the Hungarian Scientific Research Fund and the National Research and Development Fund. Nada Jabado is the recipient of a Chercheur Boursier Award from Fonds de la Recherche en Santé du Québec.

The Research Institute of the Ï㽶ÊÓƵ Health Centre (RI MUHC) is a world-renowned biomedical and health care hospital research centre. Located in Montreal, Quebec, the institute is the research arm of the MUHC, a university health centre affiliated with the Faculty of Medicine at Ï㽶ÊÓƵ. The institute supports over 500 researchers, nearly 1,000 graduate and post-doctoral students, and operates more than 300 laboratories devoted to a broad spectrum of fundamental and clinical research. The Research Institute operates at the forefront of knowledge, innovation and technology and is inextricably linked to the clinical programs of the MUHC, ensuring that patients benefit directly from the latest research-based knowledge. For further details visit: .

The NRC Biotechnology Research Institute () is located in Montreal, Quebec, and is one of the most important biotechnology R&D laboratories in Canada. More than 800 people – NRC employees, students, researchers and guest scientists – work in its three large research sectors: Health, Bioprocess, and Environment, as well as in setting up the Institute's many industrial partnerships. The Institute maintains ties with industry and universities for the benefit of Canadians.

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