People with A-T have an increased incidence (at least a 25% lifetime risk) of cancers, particularly lymphomas and leukemia, but other cancers can occur. There is currently no way to predict which individuals will develop cancer. Routine screening blood tests for leukemia and lymphoma are generally not considered helpful because there is no clear advantage in outcome from an early diagnosis. It is worthwhile, however, to consider cancer as a diagnostic possibility whenever possible symptoms of cancer arise.

In adults with A-T, very different types of cancers are seen that include cancers of the breast, esophagus, liver, colon and skin. Researchers and clinicians are still trying to determine guidelines for when breast MRI, colonoscopies or other screening tests for these types of cancers should be performed. In the meantime, every doctor caring for a person with A-T needs to be aware that all kinds of cancers can occur in people with A-T.

Treatment should avoid the use of radiation therapy, and chemotherapy drugs that work in a way that is similar to radiation therapy, as these are particularly toxic for people with A-T. The special problems of managing cancer are sufficiently complicated that treatment should be managed only in academic oncology centers after consulting with physicians who have specific expertise in A-T.

When A-T patients develop cancer, their local oncologists are encouraged to seek phone consultations from either:

Dr. Howard Lederman, Director of the A-T Clinical Center at Johns Hopkins Children’s Center at 410-955-5883 or hlederm1@jhmi.edu.

Dr. John T. Sandlund, Director of the Leukemia/Lymphoma Clinic at St. Jude Children’s Research Hospital at 901-495-3300 or john.sandlund@stjude.org.