Hepatoblastoma in families with polyposis coli
F. P. Li, W. A. Thurber, J. Seddon and G. E. Holmes
We observed hepatoblastoma in four unrelated children who have a family
history of polyposis coli and found this association in ten other kindreds
in the literature. The one child in our series who has survived
hepatoblastoma showed multiple colonic adenomas at 7 years of age. She and
eight affected maternal relatives also had congenital hypertrophy of the
retinal pigment epithelium, a new marker of gene carriers in some polyposis
coli families. These findings suggest that childhood hepatoblastoma is an
associated feature of familial polyposis coli.
