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JNCI Journal of the National Cancer Institute 1988 80(20):1626-1628; doi:10.1093/jnci/80.20.1626
© 1988 by Oxford University Press
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Journal of the National Cancer Institute, Vol. 80, No. 20, 1626-1628, December 21, 1988
© 1988 Oxford University Press

Hepatoblastoma and Familial Adenomatous Polyposis

J. E. Garber, F. P. Li, J. E. Kingston, A. J. Krush, L. C. Strong, M. J. Finegold, L. Bertario, S. BÜlow, A. Filippone, Jr., T. Gedde-Dahl, Jr., H.J. Järvinen

Dana-Farber Cancer Institute, Boston, MA and Clinical Epidemiology Branch, Division of Cancer Etiology, National Cancer Institute Bethesda, MD
Childhood Cancer Research Group, St. Bartholomew‘s Hospital London, England
The Johns Hopkins Hospital Baltimore, MD
University of Texas-M.D. Anderson Cancer Center Houston, TX
Baylor College of Medicine Houston, TX
Istituto Nazionale per lo Studio e la Cura dei Tumori Milan, Italy
University of Copenhagen Hvidovre, Denmark
Morristown Memorial Hospital Morristown, NJ
Norwegian Radium Hospital Oslo, Norway
Central Hospital Helsinki, Finland

Correspondence to: Dr. J. E. Garber, Dana Farber Cancer Institute, 44 Binney St., Boston MA 02115.

Eleven children have been identified as having hepatoblastoma and a family history of adenomatous polyposis, and 14 additional instances of this association have been collected from the literature. Among the 11 survivors of hepatoblastoma in the combined series, adenomatous lesions have been sought in seven and detected in six patients at ages 7 to 25 years. Five of these patients also have congenital hypertrophy of the rential pigment epithelium, a marker for carriers of the polyposis gene. These findings strengthen the association between hepatoblastoma and familial adenomatous polyposis and have led to the establishment of the Hepatoblastoma-Adenomatous Polyposis Registry.


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