Discovery brings new hope in the fight against cancer in children

A team of scientists from Aston University in Birmingham, UK have made a discovery that could lead to new ways of treating children who develop a particularly aggressive form of cancer. The research was funded by the Association for International Cancer Research (AICR).

Craniopharyngioma tumours cause severe headaches, vomiting, disturbed vision and growth defects. They are usually diagnosed in babies and very young children, and cause one in ten of all childhood brain tumours.

Now, new research* by Dr Eric Adams and his team at Aston University has consistently found that a gene called catenin is altered in nearly all cases studied. This they believe is important because the gene is involved with ensuring babies develop normally in the womb, and leads them to endorse the belief that craniopharyngiomas are congenital ie the defect has occurred very early in life.

’Normally, the catenin gene is switched on very briefly, carries out its duties in the cell, and then switches off at exactly the right moment. We believe that the mutations cause the gene to be switched on for far too long and this can lead to too much cell growth. It’s a bit like the accelerator button on a train getting jammed at maximum and the engine races out of control’, explains Dr Adams.

Doctors believe that when symptoms first appear the tumour is growing quite fast and this makes it very difficult to treat, even by modern surgical procedures. Because craniopharyngiomas are ’sticky’ in that they attach to surrounding brain tissue, the neurosurgeon has to be sure to remove the disease without damaging the brain. This often results in residual tumour being left behind which re-grows.

Another problem for many of these children is that the tumour grows in an area of the brain that controls the endocrine glands. Many young patients suffer from inadequate growth and delayed puberty because of hormone deficiency and require life-long hormone replacement therapy.

’We are very excited by what we have found because it means that for the first time new treatments can be developed to be directed against these gene defects. One approach might be to devise methods to knock out the defective catenin gene by adding an ’anti gene’, something that prevents the gene being switched on ­ into the diseased cells,’ Dr Adams adds.

Derek Napier, AICR’s Chief Executive believes an alternative or additional therapies to surgery is desirable, but development of new drugs or procedures depends on better understanding of how these tumours grow.

’Surprisingly, even though craniopharyngioma is a very serious disease, very few laboratory studies on its biochemistry have to date been performed. AICR has supported Dr Adams’ work for the past three years to focus on obtaining a better understanding of these tumours to help clinicians devise improved or additional treatments. I am convinced this discovery will lead to new ways to tackle what is undoubtedly a particular aggressive and debilitating disease.’