Predisposition to cancer in children and adolescents
Open accessAbstract
Childhood malignancies are rarely related to known environmental exposures, and it has become increasingly evident that inherited genetic factors play a substantial causal role. Large-scale sequencing studies have shown that approximately 10% of children with cancer have an underlying cancer predisposition syndrome. The number of recognised cancer predisposition syndromes and cancer predisposition genes are constantly growing. Imaging and laboratory technologies are improving, and knowledge of the range of tumours and risk of malignancy associated with cancer predisposition syndromes is increasing over time. Consequently, surveillance measures need to be constantly adjusted to address these new findings. Management recommendations for individuals with pathogenic germline variants in cancer predisposition genes need to be established through international collaborative studies, addressing issues such as genetic counselling, cancer prevention, cancer surveillance, cancer therapy, psychological support, and social-ethical issues. This Review represents the work by a group of experts from the European Society for Paediatric Oncology (SIOPE) and aims to summarise the current knowledge and define future research needs in this evolving field.
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Li-Fraumeni syndrome is the most prominent genetic predisposition syndrome in osteosarcoma, but hereditary retinoblastoma, helicase-associated cancers and others also contribute.
“Among all paediatric patients with cancer, those with osteosarcoma carry one of the highest rates of genetic cancer predisposition (10-20%) 12 ; Li-Fraumeni syndrome is the most prominent but hereditary retinoblastoma, helicase-associated cancers and others also contribute 157 .”
From Osteosarcoma (2022) · cites this paper as [157]
Curative osteosarcoma treatment, including anthracyclines, alkylating agents and/or topoisomerase II inhibitors, is known to cause secondary malignancies, and the risk is likely increased by individual cancer predisposition and other as yet unidentified factors.
“Curative osteosarcoma treatment, including anthracyclines, alkylating agents and/or topoisomerase II inhibitors, is known to cause secondary malignancies and the risk is likely increased by individual cancer predisposition and other as yet unidentified factors 155,157 .”
From Osteosarcoma (2022) · cites this paper as [157]
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References 124 · Cited by 129
- Authors
- Kratz, C. P., Jongmans, M. C., Cavé, H., Wimmer, K., Behjati, S., Guerrini-Rousseau, L., Milde, T., Pajtler, K. W., Golmard, L., Gauthier-Villars, M., Jewell, R., Duncan, C., Maher, E. R., Brugieres, L., Pritchard-Jones, K., & Bourdeaut, F.
- Affiliations
- Medizinische Hochschule Hannover (DE)
- University Medical Center Utrecht (NL)
- Inserm (FR)
- Innsbruck Medical University (AT)
- University of Cambridge (GB)
- German Cancer Research Center (DE)
- Université Paris Sciences et Lettres (FR)
- Chapel Allerton Hospital (GB)
- Corresponding author
- Bourdeaut, Franck
- ORCID
- Venue
- The Lancet Child & Adolescent Health
- Publisher
- Elsevier BV
- Published
- 2021-02
- Volume
- 5
- Issue
- 2
- Pages
- 142-154
- ISSN
- 2352-4642 (ISSN-L)
- OpenAlex
- W3121375769
- Cited by
- 129
- References
- 124
- Citations by year
- 2026: 19 · 2025: 24 · 2024: 30 · 2023: 25 · 2022: 20 · 2021: 10
- FWCI
- 3.13
- Type
- journal article
- Language
- en
- Open access
- green
- dspace.library.uu.nl
- Licence
- Locations
- The Lancet Child & Adolescent Health (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- Funders
- National Institute for Health and Care Research
- Bundesministerium für Bildung und Forschung (01GM1909A)
- Deutsche Kinderkrebsstiftung (A2019/26)
- NIHR Great Ormond Street Hospital Biomedical Research Centre
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › BRCA gene mutations in cancer
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genomics and Rare Diseases
- Health Sciences › Medicine › Pathology and Forensic Medicine › Genetic factors in colorectal cancer
- Keywords
- childhood cancer, cancer predisposition syndromes, germline mutations, genetic counseling, cancer surveillance, cancer prevention, pediatric oncology, psychological support, social and ethical issues
- MeSH terms
- Adolescent, Child, Female, Genetic Testing, Humans, Male, Medical Oncology, Neoplasms, Risk Assessment, Genetic Predisposition to Disease
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