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Advances in childhood eye cancer
17 January 2012
Researchers at a children's hospital in Washington have identified the genetic mechanism behind the rapid development of retinoblastoma, a rare type of childhood eye cancer. Their findings also point to a potential new way to treat the cancer, according to work published in the journal Nature.
Retinoblastoma develops in the retina - the light-sensing tissue at the back of the eye. It affects more than 5,000 children worldwide every year, many of them under the age of five.
More than nine in 10 cases can be cured if the disease is detected early enough, but the prognosis can be worse for children whose cancer is identified after it has spread beyond the eye - as is often the case in developing countries.
It has long been known that virtually all retinoblastomas are caused by damage to a gene called RB1.
However, until now scientists haven't been able to identify the subsequent steps involved in the disease's development, which can be extremely rapid.
Work is now underway to reformulate one of the experimental drugs, a SYK-inhibitor called R406, so it can be delivered directly into the eye. If successful, those efforts are expected to lead to a Phase I trial in retinoblastoma patients.
Dr Laura Bell, senior science information officer at Cancer Research UK, said the "fascinating" discovery challenged the conventional view of how retinoblastoma develops. "Even more importantly," she added, "it suggests a novel way to treat the disease".
"But further work is needed to see if this mechanism drives the development of all retinoblastomas. If confirmed, then this work could be the first step towards a new treatment that could improve survival for children with this rare type of cancer, and could help to preserve vision in children who would otherwise undergo radiotherapy or surgery."
Read full story @ Cancer Research UK
*STOP PRESS*
In conjunction with the Childhood Eye Cancer Trust (CHECT), the NHS has now published more information about retinoblastoma on the NHS Choices website. About 40-50 cases are diagnosed in the UK each year.
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