Epidemiological and familial aspects of hepatoblastoma.

Abstract

Data on the 20 cases of hepatoblastoma registered with the Manchester Children's Tumour Registry between 1954 and 1988 are presented. The cases represent about 0.6% of childhood malignancies and show a male:female ratio of 2.3:1. Age at presentation was almost always under 2 1/2 years. Cancer rates in close relatives were not significantly different from those expected. One family was confirmed as having Gardner syndrome, but the presence of other syndromes e.g., Beckwith-Wiedemann, could not be reliably detected in this retrospective series. The need to take an extensive pedigree and for clinical examination of other family members at the time of the child's diagnosis is emphasized.