Predominance of girls with cancer in families with multiple childhood cancer cases

• Main Authors: Karl-Johan Stjernfelt, Kristoffer von Stedingk, Thomas Wiebe, Lars Hjorth, Håkan Olsson, Ingrid Øra
• Format: Article
• Language: English
• Published: BMC 2017-12-01
• Series: BMC Cancer
• Subjects: Pediatric cancer; Familial cancer predisposition; Hereditary cancer syndrome; Genetic cancer susceptibility
• Online Access: http://link.springer.com/article/10.1186/s12885-017-3899-8

Abstract Background Recent studies indicate that one of four childhood cancers can be attributed to hereditary genetic abnormalities. Methods The Lund Childhood Cancer Genetic study includes newly diagnosed childhood cancer patients as well as childhood cancer survivors visiting the Department of Pediatrics or the Late Effect Clinic at Skåne University Hospital, Lund, Sweden. Questionnaires regarding family history of cancer and blood samples were provided. Reported data were validated and extended by use of the Swedish Population- and Cancer Registries. Demographics in families with one case of childhood cancer (FAM1) were investigated and compared to families with multiple cases of childhood cancer (FAM > 1) as well as to childhood cancer in the general population. Results Forty-one out of 528 families (7.8%) had more than one case of childhood cancer. In 23 families the affected children were relatives up to a 3rd degree (4.4%). In FAM > 1, 69.2% of the children with leukemia and 60% of those with tumors in the central nervous system (CNS) had a childhood relative with matching diagnosis, both significantly higher than expected. Significantly more female than male patients were observed in FAM > 1 compared to FAM1. This female predominance was most striking in childhood leukemia (77% female) and also, yet to a lesser extent, in CNS tumors (68% female). Conclusions We conclude that the high proportion of children with leukemia or CNS tumors in FAM > 1 having a childhood relative with the same diagnosis suggests a hereditary background. Moreover, we report a female predominance in childhood leukemia and childhood CNS tumors in FAM > 1, which may indicate a hereditary gender-specific risk factor in these families.