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. 2017 Dec 19;17(1):868.
doi: 10.1186/s12885-017-3899-8.

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

Karl-Johan Stjernfelt  1 , Kristoffer von Stedingk  1   2 , Thomas Wiebe  1 , Lars Hjorth  1 , Håkan Olsson  3 , Ingrid Øra  4   5
Affiliations

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

Karl-Johan Stjernfelt et al. BMC Cancer. .

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.

Keywords: Familial cancer predisposition; Genetic cancer susceptibility; Hereditary cancer syndrome; Pediatric cancer.

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Conflict of interest statement

Ethics approval and consent to participate

The study was approved by the Regional Ethics Review Board, Lund University, Sweden (no. 2008/233, 2010/231 and 2011/33). Access to the Population Registry and Cancer Registry was approved for participants and relatives.

Consent for publication

Written informed consent for publication has been acquired from all 534 study patients and/or legal guardian.

Competing interests

The authors declare no conflict of interest. The funders of the study had no role in the study design, data collection, data analysis, data interpretation, or writing of the report. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit it for publication.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Diagnosis distribution of childhood cancer cases in a Lund Childhood Cancer Genetic (LCCG) study (grey)(n = 534) compared to the childhood cancer population in the Nordic countries [23] (white). The value in brackets represents the number of study patients in the LCCG-study with corresponding diagnosis. b Families with multiple childhood cancers (FAM > 1, n = 86 in 41 families) (grey) compared to families with one childhood cancer case (FAM1, n = 487) (white) in the LCCG-study. The value in brackets represents the number of study patients and relatives with corresponding diagnosis in FAM > 1. The higher number of leukemia cases and lower number CNS cases in the LCCG cohort compared to the general population were not significant after correction for multiple testing
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