Incidence of Hepatic and Extra-hepatic Malignancies in Primary Biliary Cirrhosis (PBC)

The aim of this study was to analyze the incidence of malignancies in a large series of PBC patients from Italy. The overall sample included 178 patients (10 M, 168 F). The mean age at presentation was 52 yrs (range 29-74); 17 patients had histological stage I, 52 stage II, 66 stage III, 44 stage IV. The follow-up period ranged from 1 to 16 years (mean 5 years). During the follow-up, extra-hepatic malignancies developed in 6 cases (3.3%), and hepatocellular carcinoma (HCC) in a further 4 patients, all associated with cirrhosis (2.2%). Breast cancer developed only in one patient, resulting in a crude incidence rate of 130/100.000 person years among females. The calculated crude incidence of HCC was 492.4/100.000 person years. Three of the four patients with HCC had a superinfection with HCV. In conclusion, the incidence of breast cancer is not significantly increased. HCC has a relatively high prevalence in PBC and HCV superinfection may play an important role in favouring HCC.