Familial Cancer

, Volume 16, Issue 4, pp 605–610 | Cite as

Importance of updating family cancer history in childhood cancer survivors

  • Selena Russo
  • Meera Warby
  • Katherine M. Tucker
  • Claire E. Wakefield
  • Richard J. Cohn
Letter to the Editor


Estimates of the number of childhood cancers with a genetic basis range from 5–8.5% found in germline samples to 29% based on clinical criteria. Family history-taking practice is a fundamental first step in detecting at risk individuals and families. This study focused on Li-Fraumeni Syndrome (LFS), a highly penetrant cancer syndrome. Reported family history in a cohort of 648 of cancer survivor cohort (CCS) was examined. Eligible CCS were: (i) aged up to 14 years at diagnosis; (ii) more than 5 years postdiagnosis; (iii) treated for a childhood cancer at the study hospitals in NSW, Australia; (iv) in remission for more than 3 years. CCS completed self-administered questionnaires. Medical records confirmed diagnosis and treatment-related information. Our findings reveal an increased cancer risk among sibling and relatives of CCS. 91% of siblings diagnosed with cancer were diagnosed under the age of 40 and about 30% diagnosed under the aged of 15 revealing a 5- (RR = 5.1; 95% CI, 3.3–7.9) and 44-fold (RR = 44.6; 95% CI, 18.4–108.3) increased risked of cancer compared with the Australian population, respectively. About 2% of CCS reported that they had been diagnosed with a genetic cancer syndrome. However, 11% of survivors described a family history pattern which met Chompret criteria for screening for TP53 mutations associated with LFS. Our data suggests that familial cancer predispositions may be initially overlooked. Aperiodic and accurate ascertainment of family cancer history of childhood cancer patients and survivors is therefore recommended.


Cancer Genetic Cancer family history Oncology Pediatric Cancer syndrome Childhood cancer survivors 



The Behavioural Sciences Unit is proudly supported by the Kids with Cancer Foundation. Claire Wakefield is supported by a Career Development Fellowship from the National Health and Medical Research Council of Australia (APP1067501). This research was supported by the Kids Cancer Alliance. We would like to thank the participating hospitals, Sydney Children Hospital, Children’s Hospital Westmead and John Hunter Hospital for their collaboration and support. A special thanks to the participants and their families.

Compliance with ethical standards

Conflict of interest

No author has any conflicts of interest to disclose in relation to this manuscript.

Supplementary material

10689_2017_9988_MOESM1_ESM.docx (45 kb)
Supplementary material 1 (DOCX 44 KB)


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Copyright information

© Springer Science+Business Media Dordrecht 2017

Authors and Affiliations

  1. 1.Behavioural Sciences Unit, Sydney Children’s HospitalKids Cancer CentreRandwickAustralia
  2. 2.Discipline of Paediatrics, School of Women’s and Children’s Health, UNSW MedicineThe University of New South WalesKensingtonAustralia
  3. 3.Department of Medical Oncology, Hereditary Cancer ClinicPrince of Wales HospitalRandwickAustralia

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