Five family associations from three countries have successfully funded a research

Congreso Gliomatosis Cerebri

Behind the names of IzasFranckAnnaMathys and Nina are the stories of five children from Spain, France and the United States, who died very quickly from gliomatosis cerebri (GC). GC is a rare type of especially aggressive brain cancer that does not have a cure, and has an average life expectancy of just 14.5 months from the first symptoms.

The parents of these children created five associations, which have now raised 300,000 euros to fund the first international research project for the disease. It will take place over the next three years and involve centers from four different countries.

They are motivated to fight for a better understanding of this cancer – that spreads through the brain like a ‘spider’s web’ – on behalf of the children they’ve lost, the many others that have died from GC, and the children who might be diagnosed in the future. 

The grant was awarded as one of the initiatives that emerged after the Third International Congress on Gliomatosis Cerebri Research, held in Barcelona in 2019 with the assistance of researchers and families.

The grant will fund a project called “Targeting cellular connectivity in gliomatosis cerebri” led by English researcher Chris Jones, from The Institute of Cancer Research, London, who has been researching this glioma for more than five years. He will work in collaboration with researchers Mariella Filbin, from the Dana Farber Cancer Institute in Boston; Mara Vinci, from the Ospedale Pediatrico Bambino Gesù in Rome and David Castel, from Gustave Roussy in Paris.

Professor Chris Jones, Professor of Paediatric Brain Tumour Biology at The Institute of Cancer Research, London, explains: “Recent studies have failed to find significant differences between gliomatosis cerebri and other high-grade gliomas. Despite this, the unique clinical presentation of GC continues to be diagnosed, and patients still have an extremely poor outcome. For this reason, we have formed an international collaboration between enthusiastic researchers in London, Paris, Rome and Boston to tackle this problem with a new focus, using novel technologies to explore GC at the single cell level, in order to understand exactly what makes up these tumors, and how the individual GC cells interact with normal brain cells to produce the terrible spider’s web-like spread throughout the brain that is seen in this disease.”

Mónica Sarasa, from the Spanish association Izas, la princesa guisante explains, “eight years ago there was hardly any research or data on gliomatosis cerebri. We are proud to have been able to launch this scholarship and we are proud of the quality of the projects presented, as recognized by the evaluators. It is also a reflection of the union of the international community to promote research on gliomatosis cerebri: associations of families that are marked for the rest of their lives together with brilliant scientists who have been involved with us for years, researching and taking an active part in our conferences”.

The research project will be financed by these five associations: the Spanish Izas, la princesa guisante (key grant initiator), the French Franck, a rayon de soleilMathys, a rayon de soleil; and Nina, a rayon de soleil; and the American AYJ Fund.

There is now an international forum with more than 60 families created in 2013, but there is no official data on the number of children affected in different countries. The disease is underdiagnosed, and often confused with different encephalopathies. In Spain, for example, statistics suggest there should be about 5 cases a year that are not being diagnosed.

Statistics remind us that cancer is the leading cause of death from disease in children and adolescents, and brain tumors are the leading cause of death in children with cancer. Even so, investment in pediatric cancer research, which is different from that of adults, is very low and in the last 40 years only four treatments specific for children have been approved by the FDA (Food and Drug Administration). Most of the treatments given to children with brain tumors have not been designed for a developing brain, which often leaves survivors with considerable neurological side effects.

Izas, la princesa guisante, an association created in Huesca in 2013 after the rapid death of Izas at three years of age due to gliomatosis cerebri, has a solid set achievements since its creation. Its intense and active fight against childhood cancer, and specifically gliomatosis cerebri, has been materialized in the collaboration of the best international researchers and centers; affected families, and associations from around the world.