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Prepublished online as a Blood First Edition Paper on September 5, 2002; DOI 10.1182/blood-2002-05-1498. This Article Full Text Full Text (PDF) Erratum (v101,p2136) All Versions of this Article: 2002-05-1498v1 101/3/822    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Rosenberg, P. S. Articles by Alter, B. P. Search for Related Content PubMed PubMed Citation Articles by Rosenberg, P. S. Articles by Alter, B. P. Related Collections Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 February 2003, Vol. 101, No. 3, pp. 822-826 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Cancer incidence in persons with Fanconi anemia Philip S. Rosenberg, Mark H. Greene, and Blanche P. Alter From the Biostatistics Branch and Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD. Fanconi anemia (FA) is an autosomal recessive condition associated with congenital abnormalities, progressive pancytopenia, and a predisposition to leukemia and solid tumors. We studied a retrospective cohort of North American patients with FA. We calculated relative risks of cancer compared to the general population and cause-specific hazards of the first major adverse outcomes of FA: bone marrow transplantation (BMT) for marrow complications, acute myeloid leukemia (AML), solid tumors, or death from bone marrow failure. We also estimated the cumulative incidence of each adverse event in the presence of the competing risks. Among 145 patients with FA, 9 developed leukemia and 14 developed a total of 18 solid tumors. The ratio of observed to expected cancers (O/E ratio) was 50 for all cancers, 48 for all solid tumors, and 785 for leukemia; these increased risks were statistically significant. The highest solid tumor O/E ratios were 4317 for vulvar cancer, 2362 for esophageal cancer, and 706 for head and neck cancer. Cause-specific hazards of both death and AML peaked at 1%/y in teenage years; the hazard of BMT peaked at 4%/y at age 7. In contrast, the hazard of a solid tumor approached 8%/y by age 40 years. The cumulative incidence to age 48 was 10% for leukemia, 11% for death from marrow failure, 29% for a solid tumor, and 43% for BMT. The risk of a solid tumor may become even higher as death from aplastic anemia is reduced and as patients survive longer after BMT. © 2003 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: I. Dokal Fanconi anemia is a highly penetrant cancer susceptibility syndrome Haematologica, April 1, 2008; 93(4): 486 - 488. [Full Text] [PDF] P. S. Rosenberg, B. P. Alter, and W. Ebell Cancer risks in Fanconi anemia: findings from the German Fanconi Anemia Registry Haematologica, April 1, 2008; 93(4): 511 - 517. [Abstract] [Full Text] [PDF] N. Spardy, A. Duensing, D. Charles, N. Haines, T. Nakahara, P. F. Lambert, and S. 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