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Atypical teratoid rhabdoid tumour (AT/RT)

40% of all cancers spread to the brain

This is a very rare but fast-growing tumour of the central nervous system which usually occurs in childhood.

It can occur anywhere in the brain or the spinal cord and the symptoms vary according to its location, thus making it more difficult to diagnose.

AT/RT is responsible for around 3% of paediatric brain tumours. The tumour can be associated with mutations in a specific gene and these changes can occur spontaneously or be inherited. However, reports to date of the tumour in more than one family member are extremely rare. We don’t know what this gene does but some research is being done to find out more and identify any possible therapies.

AT/RT is a very difficult tumour to treat and, unfortunately, the outcomes remain poor. In general, older children with AT/RT tend to do better than young children. Cure rates for children over 3 are about 70%, while cure rates for children younger than 3 remain below 10%.

However, recent studies have reported that patients with newly diagnosed AT/RT had remission for 60-72 months after treatment with a specific combination of surgery, radiotherapy, and chemotherapy. Clinical trials are being carried out to assess the best combination.

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