Scientists awarded grant to investigate new drug-based treatment for NF2

Scientists from Plymouth University Peninsula Schools of Medicine and Dentistry have been awarded a grant from young person's cancer charity The Laura Crane Youth Cancer Trust to investigate a new drug-based treatment for a multi-tumour brain and nervous system cancer which affects teenagers and young adults.

The research team will work with a condition called neurofibromatosis, in particular an hereditary version called NF2.

One in 25,000 people worldwide is affected NF2, a condition where the loss of a tumour suppressor called Merlin results in multiple tumours in the brain and nervous system.

Sufferers may experience 20 to 30 tumours at any one time and such numbers often lead to hearing loss, disability and eventually death. Additional symptoms include facial pain and paralysis, numbness, speech problems, difficulty in swallowing and childhood cataracts. Many of those with NF2 develop symptoms in childhood or early adolescence, reducing their average life expectancy to just 40 years.

Currently, the only available effective therapies are repeated invasive surgery or radiotherapy aimed at one tumour at a time and which are unlikely to eradicate all the tumours in one go. NF2 can affect any family, regardless of past history, through gene mutation. There is no cure.

The research team will use non-animal methods to investigate how a cell in the brain or nervous system becomes NF2 and, with an understanding of the mechanism, look at how existing drugs can be repurposed to treat the condition and prevent multi-drug resistance.

The research will take place over two years.

The study is led by Dr. Sylwia Ammoun, Senior Research Fellow in Clinical Neurobiology at the Institute for Translational and Stratified Medicine, Plymouth University Peninsula Schools of Medicine and Dentistry. She said: "NF2 is a devastating condition which affects young people, giving them life-debilitating symptoms and the prospect of shorter than average life expectancy. It is important for scientists to address the condition so that those who suffer from it have valid and effective treatment options. We are grateful to The Laura Crane Youth Cancer Trust for this opportunity to move closer to identifying those options."

Pam Thornes, Trust Manager at the Laura Crane Youth Cancer Trust, said: "The Laura Crane Youth Cancer Trust is committed to funding vital research into cancer in young people and this is brilliantly reflected through the work of Dr Ammoun and her team. The trust is proud to be a part of such pioneering research that will investigate a new drug-based treatment for a multi-tumour brain and nervous system cancer that can offer effective treatment options for these young people."

Comments

  1. L H L H United Kingdom says:

    Hello, This is a very sincere and urgent request to please correct some essential facts in this article.
    Firstly, NF2 is NOT a cancer - in almost 100% of cases, the tumours are benign. The chance of tumours becoming malignant is no more likely in NF2 sufferers than in a normal health adult who may develop any other cancer.
    The symptoms caused by NF2 in the early stages, and the way NF2 is usually discovered and diagnosed, are due to tumours developing on both of the auditory nerves (vestibular schwannomas) which causes tinnitus and lessened hearing in one or both ears.
    Secondly, the life expectancy is NOT 40 years. The NHS website is inaccurate enough in stating that it is 65 years. The life expectancy varies enormously between individuals and in many cases is no different to any other healthy adult.
    As a 22 year old who was diagnosed with NF2 less than two months ago, it is shocking to read such inaccurate information published on a supposedly trustworthy website.
    If I had read this when I was first diagnosed and started looking online for information, I would have been much more horrified and devastated than necessary. NF2 is not a death sentence, so please don't 'advertise' it as one.
    Please, visit the Neurofibromatosis Foundation website, educate yourselves, and re-write this article. It is HORRENDOUSLY inaccurate. This is fantastic news, tainted by the rest of the article's content.
    Many thanks.

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