Researchers from St. Jude Children’s Research Hospital have reported that children with ALL who have not received radiation to the brain have normal long-term survival while irradiation is associated with the development of second neoplasms, a slight excess in mortality, and an increased unemployment rate. These findings were published in the August 14, 2003 issue of the New England Journal of Medicine.

Over the past 40 years the cure rates for childhood ALL have steadily increased. This means that a relatively large number of children with ALL will survive into adulthood and be taken care of by physicians who were not involved in their initial treatment. It is therefore important to define the long-term consequences of the intensive treatment to which these patients were subjected. For many years the primary method of preventing CNS recurrences was cranial or cranial and spinal radiation. Over the past decade it has been recognized that CNS radiation leads to long-term complications, and current CNS prophylactic regimens for childhood ALL do not include irradiation and rely on systemic and intra-thecal chemotherapy instead.

In this analysis, 856 patients who had survived 10 years or more were evaluated for medical and social adverse events. There were 597 patients who had received radiation therapy and 259 who had not. After 10 years, a total of 56 adverse events which included: death during remission (n=8), leukemic relapse (n=4), basal cell carcinoma (n=10), meningioma (n=10), malignant brain tumor (n=5), myeloid neoplasm (n=2), soft tissue sarcoma (n=2), Hodgkin’s disease (n=1), and other carcinomas (n=14). The risk of secondary cancers was 20 times higher in those receiving radiation with a cumulative incidence at 30 years from diagnosis of almost 21% versus less than 1% in un-irradiated patients. Estimated survival at 30 years from diagnosis was 98.3% for the un-irradiated patients, which was not different than the general population. Survival of irradiated patients was 95.3% which was less than for the general population. Furthermore, patients in the irradiated group had higher-than-average unemployment rates and women in the irradiated group were less likely to be married.

Comments: These data are very important for physicians taking care of ALL survivors as they identify areas where these persons are at increased risk. It is also important in that it shows that the overwhelming majority of ALL survivors live a normal life.

Reference: Pui C-H, Cheng C, Leung W, et al. Extended follow-up of long-term survivors of childhood acute lymphoblastic leukemia. New England Journal of Medicine;349:640-649.