title: Extrahepatic malignancies in primary biliary cirrhosis: a comparative study at two European centers.
abstract: Limited information and divergent results are available on the prevalence/incidence, survival, and risk factors for developing extrahepatic malignancies (EMs) in primary biliary cirrhosis (PBC). The aim of the study was to analyze the epidemiology and survival rates for EM in PBC patients. The study was conducted on two series of patients followed up at two European centers (361 in Padova, Italy, and 397 in Barcelona, Spain) for a mean 7.7 ± 7 and 12.2 ± 7 years, respectively. The cancer incidence was compared with the standardized incidence ratios (SIRs) calculated using the Cancer Registry of the Veneto Region (Italy) and the Cancer Registry of Tarragona (Spain). Seventy-two patients developed EM. The prevalence of cases was similar in Padova (9.7 %) and Barcelona (9.4 %). The overall cancer incidence was similar to the expected incidence for the general population in the same geographical area (SIR = 1.2), and so was the crude EM rate (855.01 vs 652.86 per 100,000 patient-years, respectively, RR = 1.3). Logistic regression analysis showed that advanced histological stage and extrahepatic autoimmune diseases were significantly associated with the onset of EM. Survival was similar for PBC patients with and without EM (p = n.s.), and actual survival was similar to the one predicted by the Mayo model. The incidence of EM in PBC patients was found similar in Italy and Spain and no different from that of the general population. Advanced histological stage and extrahepatic autoimmune disease were risk factors significantly associated with EM developing in PBC. The onset of cancer in PBC patients does not influence the natural history of their liver disease.
authors.affiliation: Department of Surgery, Oncology and Gastroenterology, University of Padova, Via Giustiniani, 2, Padova, Italy, annarosa.floreani@unipd.it.
authors.forename: Annarosa
authors.initials: A
authors.lastname: Floreani
authors.forename: Alice
authors.initials: A
authors.lastname: Spinazzè
authors.forename: Llorenc
authors.initials: L
authors.lastname: Caballeria
authors.forename: Ana
authors.initials: A
authors.lastname: Reig
authors.forename: Nora
authors.initials: N
authors.lastname: Cazzagon
authors.forename: Irene
authors.initials: I
authors.lastname: Franceschet
authors.forename: Alessandra
authors.initials: A
authors.lastname: Buja
authors.forename: Patrizia
authors.initials: P
authors.lastname: Furlan
authors.forename: Kenichi
authors.initials: K
authors.lastname: Harada
authors.forename: Patrick Sc
authors.initials: PS
authors.lastname: Leung
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
authors.forename: Albert
authors.initials: A
authors.lastname: Pares
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009369:Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology*; D012042:Registries; D012307:Risk Factors; D013030:Spain / Q000453:epidemiology