Cancer Research UK-funded scientists may have discovered a new way to
treat a type of childhood brain tumour that has proved incurable up
until now, according to a study published in Nature Genetics today* (Sunday).
When Cancer Research scientists at The Institute of Cancer Research, London,
studied biopsy samples from a type of childhood glioma called diffuse
intrinsic pontine glioma, or DIPG,** they found that a quarter of them
had an extremely rare genetic flaw which could be a potential new drug
target.
These findings were backed up by additional studies – also published in today’s Nature Genetics – carried out in collaboration with scientists in North America.
This flaw has not been found in any other type of cancer, but is also found in patients with a childhood development disorder known as Stone Man Syndrome, also known as fibrodysplasia ossificans progressiva, or FOP, where muscles can turn into bone.
If the genetic flaw is present in all cells of the body then a child will develop FOP, however if it only occurs in the brain then it could lead to DIPG, which is universally fatal.
Crucially, drugs are already being developed to treat this developmental syndrome, and it’s hoped that the same drugs might also help children with this rare form of brain tumour.
Study leader Dr Chris Jones, Head of the Glioma Team at The Institute of Cancer Research, London, said: "Our study has uncovered a remarkable genetic link between two very different but distressing diseases – a rare inherited condition that turns muscles into bone, and a deadly form of childhood brain cancer.
"We believe that the genetic defect linking these diseases is a potential target for cancer therapies, not least because drugs already exist against similar targets in other cancers. Understanding the link between fibrodysplasia ossificans progressiva and diffuse intrinsic pontine glioma could offer new hope to patients and their families affected by both conditions."
This particular form of glioma is a childhood brain cancer that affects between 20-30 children a year in the UK. The average survival is less than a year and there is currently no effective treatment.
Due to their location in the brain, these tumours cannot be removed by surgery, meaning the outlook for patients with DIPG is poor. On average, children with DIPG survive for less than a year and there are no effective treatments to target the disease.
That’s why Dr Jones and his team are focussed on learning more about DIPG and translating this into potential new ways to treat it.
Their latest study, which is part of our Genomics Initiative and is funded through our Catalyst Club – a pioneering venture to raise £10 million to aid research into personalising cancer treatment – is starting to piece together the genetic puzzle of DIPG.
By finding the key gene faults driving the disease, Dr Jones hopes to lay the foundations for future cures.
And how two research communities, previously unknown to each other, can now work together to widen the benefit of their research for the patients who rely on it.
Whether peering at Harry Eastlack’s overgrown skeleton, or decoding vast swathes of data from childhood tumour samples, genes are genes.
And whether research like this is aiming to stop muscle turning to bone or improve the outlook for children with brain cancer, it’s all adding to our understanding of biology and underpinning the treatments of the future.
These findings were backed up by additional studies – also published in today’s Nature Genetics – carried out in collaboration with scientists in North America.
This flaw has not been found in any other type of cancer, but is also found in patients with a childhood development disorder known as Stone Man Syndrome, also known as fibrodysplasia ossificans progressiva, or FOP, where muscles can turn into bone.
If the genetic flaw is present in all cells of the body then a child will develop FOP, however if it only occurs in the brain then it could lead to DIPG, which is universally fatal.
Crucially, drugs are already being developed to treat this developmental syndrome, and it’s hoped that the same drugs might also help children with this rare form of brain tumour.
Study leader Dr Chris Jones, Head of the Glioma Team at The Institute of Cancer Research, London, said: "Our study has uncovered a remarkable genetic link between two very different but distressing diseases – a rare inherited condition that turns muscles into bone, and a deadly form of childhood brain cancer.
"We believe that the genetic defect linking these diseases is a potential target for cancer therapies, not least because drugs already exist against similar targets in other cancers. Understanding the link between fibrodysplasia ossificans progressiva and diffuse intrinsic pontine glioma could offer new hope to patients and their families affected by both conditions."
This particular form of glioma is a childhood brain cancer that affects between 20-30 children a year in the UK. The average survival is less than a year and there is currently no effective treatment.
From bones to brains
Dr Chris Jones and his team at the ICR are scanning reams of genetic data that could provide new ways to target a variety of childhood cancers.Among these is DIPG, which affects between 20 and 30 children in the UK each year. It develops from early ‘precursor cells’ that during normal development would go on to form specialised brain cells, called ‘glial cells’.Due to their location in the brain, these tumours cannot be removed by surgery, meaning the outlook for patients with DIPG is poor. On average, children with DIPG survive for less than a year and there are no effective treatments to target the disease.
That’s why Dr Jones and his team are focussed on learning more about DIPG and translating this into potential new ways to treat it.
Their latest study, which is part of our Genomics Initiative and is funded through our Catalyst Club – a pioneering venture to raise £10 million to aid research into personalising cancer treatment – is starting to piece together the genetic puzzle of DIPG.
By finding the key gene faults driving the disease, Dr Jones hopes to lay the foundations for future cures.
Research feeds research
This study is a fascinating example of how two drastically different, but equally devastating, diseases can be brought together by the genetic events that fuel them.
And how two research communities, previously unknown to each other, can now work together to widen the benefit of their research for the patients who rely on it.
Whether peering at Harry Eastlack’s overgrown skeleton, or decoding vast swathes of data from childhood tumour samples, genes are genes.
And whether research like this is aiming to stop muscle turning to bone or improve the outlook for children with brain cancer, it’s all adding to our understanding of biology and underpinning the treatments of the future.
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