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Cancer incidence in relatives of British Fanconi Anaemia patients.
BMC Cancer 2008; 8:257BC

Abstract

BACKGROUND

Fanconi anemia (FA) is an autosomal recessive DNA repair disorder with affected individuals having a high risk of developing acute myeloid leukaemia and certain solid tumours. Thirteen complementation groups have been identified and the genes for all of these are known (FANCA, B, C, D1/BRCA2, D2, E, F, G, I, J/BRIP1, L, M and N/PALB2). Previous studies of cancer incidence in relatives of Fanconi anemia cases have produced conflicting results. A study of British FA families was therefore carried out to investigate this question, since increases in cancer risk in FA heterozygotes would have implications for counselling FA family members, and possibly also for the implementation of preventative screening measures in FA heterozygotes.

METHODS

Thirty-six families took part and data was collected on 575 individuals (276 males, 299 females), representing 18,136 person years. In this cohort, 25 males and 30 females were reported with cancer under the age of 85 years, and 36 cancers (65%) could be confirmed from death certificates, cancer registries or clinical records.

RESULTS

A total of 55 cancers were reported in the FA families compared to an estimated incidence of 56.95 in a comparable general population cohort, and the relative risk of cancer was 0.97 (95% C.I. = 0.71-1.23, p = 0.62) for FA family members. Analysis of relative risk for individual cancer types in each carrier probability group did not reveal any significant differences with the possible exception of prostate cancer (RR = 3.089 (95% C.I. = 1.09 - 8.78; Chi2 = 4.767, p = 0.029).

CONCLUSION

This study has not shown a significant difference in overall cancer risk in FA families.

Authors+Show Affiliations

Cancer Genetics Program, Departments of Human Genetics and Oncology, Sir M.B. Davis Jewish General Hospital, McGill University, Montreal, Quebec, Canada. marc.tischkowitz@mcgill.caNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18786261

Citation

Tischkowitz, Marc, et al. "Cancer Incidence in Relatives of British Fanconi Anaemia Patients." BMC Cancer, vol. 8, 2008, p. 257.
Tischkowitz M, Easton DF, Ball J, et al. Cancer incidence in relatives of British Fanconi Anaemia patients. BMC Cancer. 2008;8:257.
Tischkowitz, M., Easton, D. F., Ball, J., Hodgson, S. V., & Mathew, C. G. (2008). Cancer incidence in relatives of British Fanconi Anaemia patients. BMC Cancer, 8, p. 257. doi:10.1186/1471-2407-8-257.
Tischkowitz M, et al. Cancer Incidence in Relatives of British Fanconi Anaemia Patients. BMC Cancer. 2008 Sep 11;8:257. PubMed PMID: 18786261.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cancer incidence in relatives of British Fanconi Anaemia patients. AU - Tischkowitz,Marc, AU - Easton,Douglas F, AU - Ball,Jan, AU - Hodgson,Shirley V, AU - Mathew,Christopher G, Y1 - 2008/09/11/ PY - 2008/05/30/received PY - 2008/09/11/accepted PY - 2008/9/13/pubmed PY - 2008/10/8/medline PY - 2008/9/13/entrez SP - 257 EP - 257 JF - BMC cancer JO - BMC Cancer VL - 8 N2 - BACKGROUND: Fanconi anemia (FA) is an autosomal recessive DNA repair disorder with affected individuals having a high risk of developing acute myeloid leukaemia and certain solid tumours. Thirteen complementation groups have been identified and the genes for all of these are known (FANCA, B, C, D1/BRCA2, D2, E, F, G, I, J/BRIP1, L, M and N/PALB2). Previous studies of cancer incidence in relatives of Fanconi anemia cases have produced conflicting results. A study of British FA families was therefore carried out to investigate this question, since increases in cancer risk in FA heterozygotes would have implications for counselling FA family members, and possibly also for the implementation of preventative screening measures in FA heterozygotes. METHODS: Thirty-six families took part and data was collected on 575 individuals (276 males, 299 females), representing 18,136 person years. In this cohort, 25 males and 30 females were reported with cancer under the age of 85 years, and 36 cancers (65%) could be confirmed from death certificates, cancer registries or clinical records. RESULTS: A total of 55 cancers were reported in the FA families compared to an estimated incidence of 56.95 in a comparable general population cohort, and the relative risk of cancer was 0.97 (95% C.I. = 0.71-1.23, p = 0.62) for FA family members. Analysis of relative risk for individual cancer types in each carrier probability group did not reveal any significant differences with the possible exception of prostate cancer (RR = 3.089 (95% C.I. = 1.09 - 8.78; Chi2 = 4.767, p = 0.029). CONCLUSION: This study has not shown a significant difference in overall cancer risk in FA families. SN - 1471-2407 UR - https://www.unboundmedicine.com/medline/citation/18786261/full_citation L2 - https://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-8-257 DB - PRIME DP - Unbound Medicine ER -