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Importance of updating family cancer history in childhood cancer survivors

Familial Cancer volume 16, pages 605–610 (2017)Cite this article

Abstract

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.

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Notes

  1. Chompret’s Critera for Li Fraumeni syndrome and germline TP53 mutation screening: (i) proband with tumour belonging to LFS tumour spectrum (e.g., soft tissue sarcoma, osteosarcoma, brain tumour, premenopausal breast cancer, adrenocortical carcinoma, leukaemia, lung bronchoalveolar cancer) before age 46 years AND at least one first- or second-degree relative with LFS tumour (except breast cancer if proband has breast cancer) before age 56 years or with multiple tumors; OR (ii) Proband with multiple tumours (except multiple breast tumours), two of which belong to LFS tumour spectrum and first of which occurred before age 46 years; OR (iii) Patient with adrenocortical carcinoma or choroid plexus tumour, irrespective of family history.

References

  1. Zhang J, Walsh MF, Wu G, Edmonson MN, Gruber TA, Easton J, Hedges D, Ma X, Zhou X, Yergeau DA, Wilkinson MR (2015) Germline mutations in predisposition genes in pediatric cancer. N Engl J Med 373(24):2336–2346

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  2. Knapke S, Nagarajan R, Correll J, Kent D, Burns K (2012) Hereditary cancer risk assessment in a pediatric oncology follow-up clinic. Pediatr Blood Cancer 58(1):85–89

    Article  PubMed  Google Scholar 

  3. Kamihara J, Rana HQ, Garber JE (2014) Germline TP53 mutations and the changing landscape of Li–Fraumeni syndrome. Hum Mutat 35(6):654–662

    CAS  Article  PubMed  Google Scholar 

  4. Mai PL, Malkin D, Garber JE et al (2012) Li-Fraumeni syndrome: report of a clinical research workshop and creation of a research consortium. Cancer genet 205(10):479–487. doi:10.1016/j.cancergen.2012.06.008

    Article  PubMed  PubMed Central  Google Scholar 

  5. Baade PD, Youlden DR, Valery PC et al (2010) Trends in incidence of childhood cancer in Australia, 1983–2006. Br J Cancer 102(3):620–626. doi:10.1038/sj.bjc.6605503

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  6. Australian Institute of Health and Welfare (2015) Australian cancer incidence and mortality (ACIM) books. Authoritative information and statistics to promote better health and wellbeing (AIHW), Canberra. http://www.aihw.gov.au/acim-books. Accessed 20 January 2016

  7. Neale RE, Stiller CA, Bunch KJ, Milne E, Mineau GP, Murphy MF (2013) Familial aggregation of childhood and adult cancer in the Utah genealogy. Int J Cancer 133(12):2953–2960

    CAS  PubMed  Google Scholar 

  8. Heath JA, Smibert E, Algar EM., Dite GS, Hopper JL (2014) Cancer risks for relatives of children with cancer. J Cancer Epidemiol doi:10.1155/2014/806076

    PubMed  PubMed Central  Google Scholar 

  9. Pinto EM, Ribeiro RC, Figueiredo BC, Zambetti GP (2011) TP53-associated pediatric malignancies. Genes Cancer 2(4):485–490

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  10. Parsons DW, Roy A, Yang Y, Wang T, Scollon S, Bergstrom K, Lin FY (2016) Diagnostic yield of clinical tumor and germline whole-exome sequencing for children with solid tumors. JAMA Oncol 2(5):616–624

    Article  Google Scholar 

  11. Robson ME, Storm CD, Weitzel J, Wollins DS, Offit K (2010) American Society of Clinical Oncology policy statement update: genetic and genomic testing for cancer susceptibility. J Clin Oncol 28:893–901

    Article  PubMed  Google Scholar 

  12. Chang ET, Smedby KE, Hjalgrim H, Glimelius B, Adami HO (2006) Reliability of self-reported family history of cancer in a large case-control study of lymphoma. J Natl Cancer Inst 98(1):61–68. doi:10.1093/jnci/djj005

    Article  PubMed  Google Scholar 

  13. Ferrante JM, Ohman-Strickland P, Hahn KA, Hudson SV, Shaw EK, Crosson JC, Crabtree BF (2008) Self-report versus medical records for assessing cancer-preventive services delivery. Cancer Epidemiol Prev Biomark 17(11):2987–2994

    Article  Google Scholar 

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Acknowledgements

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.

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Affiliations

  1. Behavioural Sciences Unit, Sydney Children’s Hospital, Kids Cancer Centre, Level 1 South, Sydney Children’s Hospital, High Street, Randwick, NSW, 2031, Australia

    Selena Russo, Claire E. Wakefield & Richard J. Cohn

  2. Discipline of Paediatrics, School of Women’s and Children’s Health, UNSW Medicine, The University of New South Wales, Kensington, NSW, Australia

    Selena Russo, Claire E. Wakefield & Richard J. Cohn

  3. Department of Medical Oncology, Hereditary Cancer Clinic, Prince of Wales Hospital, Randwick, Australia

    Meera Warby & Katherine M. Tucker

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  1. Selena Russo
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  2. Meera Warby
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  3. Katherine M. Tucker
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  4. Claire E. Wakefield
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Correspondence to Selena Russo.

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Russo, S., Warby, M., Tucker, K.M. et al. Importance of updating family cancer history in childhood cancer survivors. Familial Cancer 16, 605–610 (2017). https://doi.org/10.1007/s10689-017-9988-7

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  • DOI: https://doi.org/10.1007/s10689-017-9988-7

Keywords

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