547 CHOLANGIOCARCINOMA IN ITALY: A NATIONAL SURVEY ON DEMOGRAPHIC AND CLINICAL CHARACTERISTICS, DIAGNOSTIC MODALITIES AND TREATMENT

We report the results of a survey promoted in Italy by the AISF“Cholangiocarcinoma” (CCA) committee aimed to evaluate the demographic and clinical characteristics of IH(intra-hepatic)- and EH(extra-hepatic)-CCA. Members of AISF (Italian Association Liver Disease) and SIGE (Italian Society of Gastroenterology) were asked to fill out a questionnaire describing CCA patients recruited in the last 12 months. 218 consecutive incident CCA cases were analyzed. 47% were EH-CCA and 53% IH-CCA with an age higher for EH-CCA. A history of past smoking, cirrhosis, viral cirrhosis and positivity for viral hepatitis markers were more frequent in IH-CCA than EH-CCA. The clinical onset of the disease was earlier for IH-CCA than EH-CCA. An incidental asymptomatic presentation occurred in 28% of IH-CCA vs 4% EH-CCA while 74% EH-CCA presented with jaundice vs 28% IH-CCA. Abdominal pain without other symptoms was more frequent in IH-CCA (19.8 vs 4%). Of the 116 IH-CCA cases, 91% presented as single intra-hepatic mass, while 50% EH-CCA was peri-hilar. Diagnosis was time- and cost-consuming since 70% of all CCA cases received at least 3 different imaging procedures. Tissue- proven diagnosis was obtained in 80% of cases. As far as treatment was concerned, 57% of IH-CCA and 42% of EH-CCA received opensurgery. However, surgery was considered curative in 50/116 IH- CCA (43%) vs 17/102 (17%) EH-CCA. 18% of IH- vs 4% of EH-CCA received no treatment. In conclusion, in Italy IH-CCA is managed as frequently as EH-CCA. In comparison with EH-CCA, IH-CCA occurs at younger age, is more frequently associated with smoking habits, cirrhosis and/or hepatitis virus infection and with an incidental asymptomatic presentation. In contrast, most EH-CCA are jaundiced at the diagnosis. CCA diagnostic management is cost- and time- consuming with a curative surgical treatment applicable in 43% IH-vs 17% EH-CCA.