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Karen Curtin, Ken R. Smith, Alison Fraser, Richard Pimentel, Wendy Kohlmann and Joshua D. Schiffman Familial risk of childhood cancer and tumors in the li-fraumeni spectrum in the utah population database: Implications for genetic evaluation in pediatric practice International Journal of Cancer 133

Version of Record online: 12 JUN 2013 | DOI: 10.1002/ijc.28266

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Childhood cancer, although relatively rare compared to adulthood cancers, is the leading cause of death in children up to 14 years of age. Little is known about the hereditary components of the disease. Using a large population-based genealogical database in the US state Utah, Curtin and colleagues report that children with cancer have an increased risk for positive cancer family history as compared to controls. Siblings of patients diagnosed before age five have a 3.6-fold childhood cancer risk. Notably, a second report in this issue of IJC also investigated risks associated with childhood cancer and shows an increased risk of adulthood cancer in relatives of children with cancer (Neale et al.). A resulting clinical recommendation is that a three-generation family history be collected and updated for all pediatric patients, and that families with clusters of several cancers are referred to genetic counseling.

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