Risk of Hepatoblastoma in Familial Adenomatous Polyposis
Document Type
Article
Publication Title
American Journal of Medical Genetics
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.
DOI
10.1002/ajmg.1320430621
Publication Date
8-1-1992
ISSN
1096-8628
Recommended Citation
Hughes LJ, Michels VV. Risk of Hepatoblastoma in Familial Adenomatous Polyposis. American Journal of Medical Genetics. 1992; 43(6). doi: 10.1002/ajmg.1320430621.
