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Gliomatosis cerebri research in children and young adults

My laboratory at the Institute of Cancer Research studies paediatric brain tumours. Where we and many other groups around the world have recently made enormous strides in unravelling the underlying biology of these diseases. There remain. however, several types of cancer in the brain about which we know very little. One of these is gliomatosis cerebri (GC), a rare brain tumour that mostly affects children and young adults. II is a particularly difficult tumour to treat. as it spreads rapidly throughout the entire brain, and currently we have no effective drugs against ii. Almost nothing is known about What drives these tumours, so we have undertaken an international collaboration to study GC in order to identify new ways In which we might treat these patients.

On behalf of the European Society of Paediatric Oncology (SIOPE) High Grade Glioma Biology Group, we have begun to collect these rare cases of GC from children and young adults from across Europe. As a similar exercise is also underway in North America, at a recent working group meeting in Paris we have agreed to coordinate effort in a truly world-wide consortium. Our intention Is to characterise these precious tumour specimens in as comprehensive a way as possible. We are able to fully sequence the entire genetic code of these tumours. and for the first lime are beginning to build up a picture of the molecular characteristics that define the disease. We have already identified certain genetic alterations that may be targetable by available drugs which might be tested in GC patients. A dose relationship with clinical colleagues means that this information is shared rapidly such that future clinical trials can be designed on the basis of the new biological information we are generating. This part of the work has been supported by the Rudy A Menon Foundation and initial seed funding from the Royal Marsden Hospital.

It is becoming dear that GC falls into the wider group of tumours known as gliomas. and represents a particularly invasive variant of the diseases. Ongoing work in our laboratory is trying to compare GC samples with other gliomas to understand what causes this extraordinarily diffuse spread of cancer cells. We are working with colleagues in France and Spain to collect. fresh GC specimens such that we may attempt to grow these cells in the laboratory in order to study how they move and work out strategies to halt this. Developing these models is also fundamental to test Whether novel drug treatments will be effective - currently there are no known GC models available worldwide. As part of our international collaboration. we are working to together to address this, and to share any resources we are able to develop to other researchers.

Working on such a rare disease has its challenges. but with no suitable treatments available and such a poor clinical outcome. the international community of paediatric brain tumour researchers has come together to tackle GC as a priority. We hope that by applying the latest molecular techniques we will be able to repeat the advances we are making in other childhood brain tumours and come up with new insight into how to treat children and young adults with GC.

Prof. Chris Jones, Institute of Cancer Research

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