Abstract
BackgroundThere are few reports of the association of other cancers with Ewing sarcoma in patients and their relatives. We use a resource combining statewide genealogy and cancer reporting to provide unbiased risks.MethodsUsing a combined genealogy of 2.3 million Utah individuals and the Utah Cancer Registry (UCR), relative risks (RRs) for cancers of other sites were estimated in 143 Ewing sarcoma patients using a Cox proportional hazards model with matched controls; however, risks in relatives were estimated using internal cohort‐specific cancer rates in first‐, second‐, and third‐degree relatives.ResultsCancers of three sites (breast, brain, complex genotype/karyotype sarcoma) were observed in excess in Ewing sarcoma patients. No Ewing sarcoma patients were identified among first‐, second‐, or third‐degree relatives of Ewing sarcoma patients. Significantly increased risk for brain, lung/bronchus, female genital, and prostate cancer was observed in first‐degree relatives. Significantly increased risks were observed in second‐degree relatives for breast cancer, nonmelanoma eye cancer, malignant peripheral nerve sheath cancer, non‐Hodgkin lymphoma, and translocation sarcomas. Significantly increased risks for stomach cancer, prostate cancer, and acute lymphocytic leukemia were observed in third‐degree relatives.ConclusionsThis analysis of risk for cancer among Ewing sarcoma patients and their relatives indicates evidence for some increased cancer predisposition in this population which can be used to individualize consideration of potential treatment of patients and screening of patients and relatives.
Highlights
Ewing sarcoma is a rare tumor occurring most often in the bones or soft tissues of children and young adults
Cancers of four different primary cancer sites other than Ewing sarcoma were observed in at least one Ewing sarcoma case. For those Ewing sarcoma patients where data were available for age at diagnosis for both cancers, 50% of the other cancers occurred at an average of 18 years after the diagnosis of Ewing sarcoma, and 50% of the other cancers occurred at an average of 2 years before the diagnosis of Ewing sarcoma
relative risks (RRs) estimates for cancers that were observed in significant excess among first, second, and third‐degree relatives of the 143 Ewing Sarcoma patients are summarized in Table 2; TABLE 1 Estimated hazard ratios for cancers observed in significant excess in the 143 individuals diagnosed with Ewing sarcoma
Summary
Ewing sarcoma is a rare tumor occurring most often in the bones or soft tissues of children and young adults. Novakovic[23] reported no overall increased risk of cancer among 4628 first‐ or second‐degree relatives of 256 Ewing sarcoma patients; several specific tumor types were observed in significant excess, including stomach cancer (RR = 2.0; 1.4, 2.8); melanoma (RR = 1.9; 1.2, 2.8); brain tumor (RR = 1.9; 1.1, 3.0); and bone cancer (RR = 4.2; 1.7, 8.6). Risks of these cancers were higher among maternal than paternal relatives, but not significantly. We utilize a unique Utah population‐based resource to expand the characterization of risk for cancers of other sites in Ewing sarcoma patients and in their relatives
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