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Article
May 8, 1987

Hepatoblastoma in Families With Polyposis Coli

Author Affiliations

From the Clinical Studies Section, Clinical Epidemiology Branch, National Cancer Institute, Bethesda, Md (Dr Li); the Dana-Farber Cancer Institute (Dr Li and Ms Thurber), The Children's Hospital (Ms Thurber), and Massachusetts Eye and Ear Infirmary (Dr Seddon), Boston; and the University of Kansas Medical Center, Kansas City (Dr Holmes).

From the Clinical Studies Section, Clinical Epidemiology Branch, National Cancer Institute, Bethesda, Md (Dr Li); the Dana-Farber Cancer Institute (Dr Li and Ms Thurber), The Children's Hospital (Ms Thurber), and Massachusetts Eye and Ear Infirmary (Dr Seddon), Boston; and the University of Kansas Medical Center, Kansas City (Dr Holmes).

JAMA. 1987;257(18):2475-2477. doi:10.1001/jama.1987.03390180093030
Abstract

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.

(JAMA 1987;257:2475-2477)

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