title: Risk of hepatoblastoma in familial adenomatous polyposis.
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 less than 1% for hepatoblastoma can be cited to persons with FAP for their children.
authors.affiliation: University of Health Sciences, College of Osteopathic Medicine, Kansas City, Missouri.
authors.forename: L J
authors.initials: LJ
authors.lastname: Hughes
authors.forename: V V
authors.initials: VV
authors.lastname: Michels
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000235:genetics*; D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology / Q000235:genetics*; D005260:Female; D017491:Genes, APC; D006579:Heterozygote; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000453:epidemiology / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012189:Retrospective Studies; D012307:Risk Factors