Risk of hepatoblastoma in familial adenomatous polyposis

Authors

  • Linda J. Hughes,

    1. Visiting Mayo Medical School Clerkship, the University of Health Sciences, College of Osteopathic Medicine, Kansas City, Missouri
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  • Dr. Virginia V. Michels

    Corresponding author
    1. Department of Medical Genetics, Mayo Clinic, Rochester, Minnesota
    • Department of Medical Genetics, Mayo Clinic, Rochester, MN 55905
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Abstract

Infantile and childhood hepatoblastoma occurs more frequently in persons heterozygous for the familial adenomatous polyposis (FAP) gene than in the general population. This observation is based on numerous case reports plus the results of an international survey of FAP registries. However, the frequency of this rare tumor in FAP patients is unknown. In a retrospective review of our family history data, 2/470 (0.42%) children born to 241 patients with FAP had hepatoblastoma. This figure is significantly higher than the 1/100,000 incidence of hepatoblastoma in the general population. However, for genetic counseling purposes, an empiric risk of <1% for hepatoblastoma can be cited to persons with FAP for their children. © 1992 Wiley-Liss, Inc.

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