Treating cancer in A-T: the way ahead

In late October a group of experts on cancer in A-T gathered together in Boston for a two-day workshop. Organised by the A-T Children's Project, the workshop entitled Optimizing Cancer Management in A-T focused on bringing together our current knowledge about cancer in people with A-T and identifying what needs to be done to improve care and outcomes for these people.Boston and Charles River

It is well known that people with A-T have an increased incidence of cancer. In the UK it has been calculated that around 22% of people with A-T develop cancer. These are predominantly lymphoid cancers, such as lymphoma and certain types of leukaemia, though in adult women with A-T the risk of breast cancer is significant.

However, A-T also brings complications in treating cancer. In particular, sensitivity to radiation means that neither radiotherapy nor so called radio-mimetic drugs, which imitate the effects of radiation, can be used. Weakened immune systems and a propensity to lung problems can also complicate the treatment of cancer in people with A-T.

As cancer tends to be treated locally, few clinicians have seen more than one or two patients with A-T and cancer, and events like this, where experiences can be shared and discussed are of vital importance.

Participants discussed a wide range of issues, including:

  • the types of cancers seen in patients with A-T
  • the advantages and disadvantages of aggressive versus less aggressive treatment protocols (a protocol is effectively a treatment plan)
  • supportive care and adverse reactions to chemotherapy
  • the challenges associated with establishing new treatment protocols and clinical trials for cancer and A-T
  • new directions for research.

 The outcomes of the meeting were many and varied. These will be gathered together and published as a paper, which will summarise what is currently known about cancer in A-T and what needs to be done to improve our knowledge and treatments. But amongst the points of agreement were:

  • The need to pool data on cancer in people with A-T and ideally to establish an international registry
  • While cancers associated with A- T can be quite robustly treated, there is need for vigilance, particularly with regard to infections, cystitis and neuropathies
  • A-T is a variable condition and this may affect diagnosis, treatment and outcomes
  • It is important to get a proper baseline picture at the start of therapy, with assessments of immunological status, lung health, state of nutrition perhaps an MRI scan
  • This data, along with better pathology studies and perhaps a central tissue bio-bank would be very helpful in better understanding cancer in A-T
  • We should produce treatment guidelines for supportive care and some specific treatment protocols, starting with the most common cancers, such as B-cell lymphomas

Organisation and preparation of the paper will be led by the two co-chairs of the workshop, John “Torrey” Sandlund from St. Jude Children’s Research Hospital and Douglas Weckstein, from the Dana-Farber Cancer Institute.

The A-T Children's Project deserves our thanks and congratulations for having organised this meeting. It is a major step forward to have brought together this group of experts and to have gained such a strong consensus on the way ahead.

Thanks to experts such as Prof. Malcolm Taylor at the University of Birmingham and  Dr Louise Izatt at Guy’s and St Thomas’, a great deal of knowledge and expertise exists here in the UK regarding the incidence and genetics of cancer. However, despite this wealth of knowledge, there was no oncologist to represent the UK at this workshop. However, the A-T Society is currently talking to staff at the two specialist centres and beyond to agree how we can build up a similar expertise in the treatment of cancer.

 

We are very grateful to the A-T Children's Project for  providing much of the information used in this article