A TEAM OF Irish researchers at Trinity College Dublin made a major breakthrough in the fight against neuroblastoma, a childhood cancer that targets ten children in Ireland every year. It is believed that the new findings will result in improved treatments for the disease.
Neuroblastoma is the most common cancer in children aged under two years and affects the nervous system. Neuroblastoma is a cancer of special nerve cells called neuroblasts. These can appear in nervous tissue on areas of the body such as the abdomen, chest and spine. The disease accounts for 15 percent of cancer deaths in children.
The research, conducted in collaboration between the Ludwig Institute for Cancer Research Stockholm and Trinity College Dublin, headed up by Dr Adrian Bracken, looked closely at the role of the CHD5 tumor suppressor during normal nervous system development.
The CHD5 gene is often inactive in the most severe forms of neuroblastoma, meaning that the immature cells known as neuroblasts fail to develop and can become cancer cells.
The team discovered that CHD5 is required for the cellular transition from a stem cell to a mature neuron and investigated how the CHD5 operates.
Ludwig Institute for Cancer Research Stockholmn said that the research could lead to new ways to treat neuroblastoma, perhaps using currently approved drugs. Treatment for neuroblastoma can be very aggressive. One treatment is retinoic acid, a drug that can drive neuronal maturation.
Unresponsiveness to retinoic acid can be common in more malignant CHD5-negative neuroblastoma cells, said Johan Holmberg of the Ludwig Institute, but added that if CHD5 could be re-activated in such hard-to-treat patients and increase responsiveness to retinoic acid.
Commenting on the study, Dr Adrian Bracken said:
Understanding the role of genes whose deletion or inactivation is associated with disease is central to designing intelligent therapeutic strategies. Our work has unravelled the normal function of the CHD5 gene, and suggests that its inactivation in neuroblastoma leads to an inability of these cells to correctly mature or differentiate. Our future work will assess the potential benefit of reactivating CHD5 in neuroblastoma cells which usually retain one silenced copy of this gene. We hope that this research will lead to new and improved treatments for children with this disease.
News of breakthroughs in neuroblastoma is greeted with a cautious welcome from families with children who are suffering from the disease, like the family of Robyn Smyth from Dublin. Robyn Smyth is eight years old and has been battling neuroblastoma for most of her life.
When Robyn was three she was first diagnosed with the illness. She underwent chemotherapy, radiotherapy, a stem cell transplant and was on tablets for many months. She has been in remission for nearly four years, but the illness has returned. She is currently undergoing chemotherapy.
Speaking to TheJournal.ie, Robyn’s mother Bernadette Smyth said:
Robyn is in the middle of her chemo now. All we can do now is wait to see how she responds to the treatment. She will be getting a scan soon, so we will just have to wait. While I welcome any new research or breakthroughs to do with neuroblastoma, it usually takes years for any new treatments to be introduced.New treatments are desperately needed when it comes to neuroblastoma. The treatment is very aggressive for children. Robyn knows she is sick, but she doesn’t understand how serious it is.
She added that if Robyn is unresponsive to her treatment, they hope to travel to the US to the Memorial Sloan-Kettering Cancer Centre in New York to get treatment. The family are currently fundraising money for Robyn.
For more information on Robyn and how to donate to her cause please visit Robynslife.com. There will also be a collection for Robyn during the Bohs versus Shamrock Rovers Airtricity league match at Dalymount Park on Friday next.
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