Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Childhood Cancer and Pediatric Cancer Predisposition Syndromes

  • Stefan K. Zöllner
  • Jeffrey A. Toretsky
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_7234-1

Definition

Genetic factors play a crucial role in the development of cancer. There are several genetic conditions which are associated with increased risk of cancers, termed hereditary cancer syndromes. In adults, the percentage of cancer attributed to underlying, inherited genetic mutations is estimated at 5–10 %. However, the rate of cancer predisposition in children might be significantly higher, and potentially as high as a third of all new pediatric cancer diagnoses may be due to an inherited genetic cause. Identification of at-risk individuals leads to early tumor detection and better prognosis for some cancers. Familial neoplastic syndromes, bone marrow failure syndromes, inherited immunodeficiency (see Immunosuppression and Cancer), and numerical chromosome abnormalities are presented.

Characteristics

Hereditary Cancer Predisposition Syndromes

Although hereditary cancer predisposition syndromes are rare, the list of well-defined inherited cancer predisposition syndromes is...

Keywords

Tuberous Sclerosis Complex Multiple Endocrine Neoplasia Type Fanconi Anemia Testicular Germ Cell Tumor Adenomatous Polyposis Coli Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Alter BP et al (2010) Malignancies and survival patterns in the National Cancer Institute inherited bone marrow failure syndromes cohort study. Br J Haematol 150(2):179–188PubMedPubMedCentralGoogle Scholar
  2. Garber JE, Offit K (2005) Hereditary cancer predisposition syndromes. J Clin Oncol 23(2):276–292CrossRefPubMedGoogle Scholar
  3. Schiffman JD et al (2013) Update on pediatric cancer predisposition syndromes. Pediatr Blood Cancer 60(8):1247–1252CrossRefPubMedGoogle Scholar
  4. Shapiro RS (2011) Malignancies in the setting of primary immunodeficiency: Implications for hematologists/oncologists. Am J Hematol 86(1):48–55CrossRefPubMedGoogle Scholar
  5. Stiller CA (2004) Epidemiology and genetics of childhood cancer. Oncogene 23(38):6429–6444CrossRefPubMedGoogle Scholar
  6. Strahm B, Malkin D (2006) Hereditary cancer predisposition in children: genetic basis and clinical implications. Int J Cancer 119(9):2001–2006CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Pediatric Hematology and OncologyUniversity Childrens Hospital MünsterMünsterGermany
  2. 2.Department of Oncology and Pediatrics, Lombardi Comprehensive Cancer CenterGeorgetown UniversityWashingtonUSA