Patients with primary biliary cirrhosis (PBC) may be at increased risk for
malignancies. Several studies have addressed the risk of specific malignanc
ies; however, there is little information about overall incidences of malig
nancies in these patients. We hypothesize that these patients may be at an
increased risk for cancer. We performed a retrospective chart review evalua
ting patients with the diagnosis of PBC and malignancies. We reviewed recor
ds of patients with PBC presenting to the Mayo Clinic between 1976 and 1985
. The diagnosis of PBC was made using evidence of cholestasis, positive ant
imitochondrial antibody titers and liver biopsy findings consistent with PB
C, The incidence of malignancies were then compared with published data by
the Surveillance, Epidemiology and End Results (SEER) program of the Nation
al Cancer Institute. Of the 1,692 patients with PBC in the Mayo Clinic data
base, 114 patients were identified with primary cancer,The number of malig
nancies was higher than would be anticipated by chance alone; with 93 obser
ved versus 62.4 expected events (P < .001), Hepatobiliary malignancies had
a relative risk of 46 (P < .0001) for women and SS (P < .0001) in men, Ther
e was a dramatic increased risk for development of hepatobiliary malignanci
es. PBC patients might benefit from more aggressive surveillance for hepato
biliary malignancies during their lifetime.