INCIDENCE AND RISK FACTORS FOR EXTRA-HEPATIC MALIGNANCIES (EM) IN PRIMARY BILIARY CIRRHOSIS: A COMPARATIVE STUDY FROM TWO EUROPEAN REFERRAL CENTERS

Background and Aims: There is limited information on the prevalence/incidence, survival, and risk factors for developing EM in PBC. Aim: To analyze the incidence/prevalence, risk factors and survival for EM in patients with PBC in two European centers. Methods: The study was carried out in two series of PBC patients (361 of Padova, Italy and 397 of Barcelona, Spain) followed-up for a mean period of 7.7±7 and 12.2±7 years respectively. The incidence of EM was compared to the estimated incidence data from IARC. Demographic features and factors associated with tumor development were recorded. Survival analysis was compared with the expected survival predicted by the Mayo model. Results: 72 patients developed EM. The prevalence of cancer was similar in Padova (9.7%) and Barcelona (9.4%). The incidence of EM was also similar (855.01 vs 652.86, p=n.s.). The overall incidence of EM in the study population was similar to the expected incidence in the same geographical area (observed/expected ratio = 1.18). Older age was the only factor associated with the development of EM. Familial predisposition was associated with higher likelihood of EM in Padova series. Observed survival of patients was similar in those with either or without EM (29.2 and 33.4 years, p=n.s.) and wa similar to that predicted by the Mayo model. Conclusions: The prevalence/incidence of EM is similar in Italy and Spain and not different from the general population. Older age is the only risk factor associated with EM. The occurrence of cancer during the follow-up does not influence the natural history of liver disease.