Only about 5% of childhood cancers have a clear hereditary basis. From a UK study of 16,564 children with cancer it was estimated that 4% of childhood cancers had a known genetic-hereditary basis (Narod, 1991). The most frequently recorded diagnoses
with a hereditary basis were: retinoblastoma (37% of retinoblastoma cases), Wilms' tumour (7% of cases), leukaemia (3% of cases) - associated with Down's syndrome, and brain tumours (2% of cases) - associated with neurofibromatosis. Similarly, in a US study of 556 twins with childhood cancer (Buckley 1996) the overall rate of inherited cancers was 5% and just 2% when excluding retinoblastoma (based
on MZ case-wise concordance rates). Also, rates of cancer among off-spring of childhood cancer survivors are reported to be similar to those for the general population. Future studies will determine if small differences
in specific genes (polymorphisms) are associated with cancer susceptibility in the context of gene-environment interactions.
Narod SA, et al. An estimate of the heritable fraction of childhood cancer. Br J Cancer 1991 Jun;63(6):993-9 Related articles (PubMed)
Buckley JD, et al. Concordance for childhood cancer in twins. Med Pediatr Oncol 1996 Apr;26(4):223-9 Related articles (PubMed)
Medline Search: hereditary childhood cancer (PubMed)
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Syndromes Predisposing to Childhood Cancer
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Clericuzio CL Recognition and management of childhood cancer syndromes: a systems approach. [Review] Am J Med Genet 1999 Jun 25;89(2):81-90 Related articles (PubMed)
Shannon K. Genetic predispositions and childhood cancer. [Review] Environ Health Perspect 1998;106 Suppl 3:801-6 Related articles (PubMed)
Narod SA, et al. Congenital anomalies and childhood cancer in Great Britain. Am J Hum Genet 1997 Mar;60(3):474-85 Related articles (PubMed)
Narod SA, et al. An estimate of the heritable fraction of childhood cancer. Br J Cancer 1991 Jun;63(6):993-9 Related articles (PubMed)
Medline Search: childhood cancer AND syndrome AND risk (PubMed)
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Risk of Cancer Among Offspring of Childhood-Cancer Survivors
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Sankila R, et al. Risk of cancer among offspring of childhood-cancer survivors. Association of the Nordic Cancer Registries and the Nordic Society of Paediatric Haematology and Oncology. N Engl J Med 1998 May 7;338(19):1339-44 Related articles (PubMed)
Hawkins MM, et al. Cancer in the offspring of survivors of childhood leukaemia and non-Hodgkin lymphomas. Br J Cancer 1995 Jun;71(6):1335-9 Related articles (PubMed)
Hawkins MM, et al. Cancer among 1,348 offspring of survivors of childhood cancer. Int J Cancer 1989 Jun 15;43(6):975-8 Related articles (PubMed)
Medline Search: cancer AND offspring of childhood cancer survivors (PubMed)
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