In a retrospective review of 85 patients with primary biliary cirrhosis (PBC), 10 (11.8%) were noted to have extrahepatic malignant neoplasm. In seven female patients the tumour developed within a mean of 3.5 yr after the clinical onset of PBC. This observed number of tumours, 3.5 times more common than the expected age-adjusted incidence, was statistically significant at the 0.5% level.
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