AT and cancer
People with AT have a greatly increased risk of developing cancers. In the United Kingdom the lifetime probability for a person with AT to develop cancer appears to be to be around 22%. However this includes many people with variant AT and the risk for people with classic AT is probably around 27%.
A study carried out in the UK and the Netherlands* showed that up to the age of 16, the cancers were predominantly lymphoid cancers, for example lymphomas or lymphoid leukaemias. Over the age of 16, cancers were fairly evenly divided between lymphoid and other cancers. The most common non-lymphoid cancer was breast cancer, which was found exclusively in women over the age of 16, but others included non-lymphoid-leukaemias, brain tumours and endocrine cancers. In the 12 months to February 2018, three cases of oesophageal cancer were diagnosed.
Cancers in AT can strike at any age, though there is evidence that they are less likely in childhood for people with variant AT. Adult women with AT have a high risk of developing breast cancer and a special screening programme has been set up for them. From the age of 25, they should be screened annually with an MRI. More information on this screening programme is available here.
One major issue in treating cancers in people with AT is that radiotherapy cannot be used due to the radio-sensitivity the condition brings. It is also inappropriate to use drugs that mimic the effects of radiation. As cancers are usually treated locally rather than in the national centres, and vary considerably in their type, it has taken a longer time to build up clinical expertise in treating them.
Today there is a growing knowledge and experience in the treatment of cancers in AT. Historically, people with AT have tended to be treated with less intensive treatment regimes than those who do not have the condition. However, while certain aspects of the regime may have to be modified, there is no evidence that people with AT cannot tolerate intensive therapy.
Anyone with AT who is diagnosed with cancer is advised to contact our Support Team Kay Atkins (Head of Services) or Anne Murray (Advocacy and Support Manager) on 01582 760733 or email support. Kay or Anne will ensure that their doctors are put in touch with colleagues in the UK or abroad who have experience of treating people with AT.
There is more information on treating cancer in the clinical guidance document published by the A-T Society in October 2014.
Statement on the increased risk of cancer in people with ataxia-telangiectasia
* Reiman et al, Br J Cancer 2011, 105: 586-591. This paper can be read on-line at http://www.nature.com/bjc/archive/.