Low risk of solid tumors in persons with Down syndrome

Abstract

Purpose: The aim of this study was to investigate cancer incidence in a large cohort of persons with Down syndrome.Methods: Down syndrome was identified from the Danish Cytogenetic Register. Cancer occurrence was identified by linkage to the Danish Cancer Registry. Standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) were calculated based on observed and expected numbers from rates for all Danish residents. The cohort consisted of 3,530 persons with Down syndrome contributing 89,570 person-years at risk.Results: Acute leukemia risk was highest from 1-4 years of age and remained elevated until age 30. The overall risk of solid tumors was decreased (SIR 0.45; 95% CI 0.34-0.59), especially in persons 50 years or older (SIR 0.27; 95% CI 0.16-0.43). We found a significantly lower risk of lung cancer (SIR 0.10; 95% CI 0.00-0.56), breast cancer (SIR 0.16; 95% CI 0.03-0.47), and cervical cancer (SIR 0.0; 95% CI 0.00-0.77). Testicular cancer was the only solid tumor with an increased SIR (2.9; 95% CI 1.6-4.8). Conclusions:The risk of all major groups of solid tumors was decreased, except testicular cancer. Altered screening strategies should be considered for persons with Down syndrome. This unusual pattern of cancer occurrence may help understanding carcinogenesis in the general population.

Long‐term sequelae in survivors of childhood leukemia with Down syndrome: A childhood cancer survivor study report

“…The lower risk for SMNs may reflect the established fact that children and adults with DS are actually at lower risk of solid tumors compared with the general population . People with DS are at significantly lower risk of breast, lung, and cervical cancer . The lower risk of second cancers in DS survivors may indicate a potential protective effect of 1 or more chromosome 21‐localized genes in reducing the risk of secondary cancers.…”

Late mortality and morbidity among long‐term leukemia survivors with Down syndrome: A nationwide population‐based cohort study

“…The first systematic survey of the association between leukemia and DS was published in 1957 . The cumulative risk of leukemia for individuals with DS at the age of 30 years is 2.5% . Individuals with DS constitute 2% of all acute lymphoblastic leukemia (ALL) cases and 14% of all acute myeloid leukemia (AML) cases .…”

Red blood cell metabolism in Down syndrome: hints on metabolic derangements in aging

“…For example, rates of most solid malignancies are lower among people with DS, yet they are highly predisposed to develop Alzheimer's disease (AD), several autoimmune disorders, leukemia, pulmonary hypertension, and various hearing and vision problems. [8][9][10][11][12][13] Some of the more prevalent comorbidities could be explained by the altered dosage of expression of genes encoded on chromosome 21, such as amyloid protein or interferon receptors, factors likely contributing to the early onset of AD and autoimmune disorders.…”