title: Cholangiocarcinoma in Italy: A national survey on clinical characteristics, diagnostic modalities and treatment. Results from the "Cholangiocarcinoma" committee of the Italian Association for the Study of Liver disease.
abstract: BACKGROUND
  Very few studies assessed cholangiocarcinoma clinical characteristics.
  
  
  OBJECTIVE
  To evaluate the clinical characteristics of intra-hepatic (IH) and extra-hepatic (EH)-CCA.
  
  
  METHODS
  We performed a national survey based on a questionnaire.
  
  
  RESULTS
  218 cholangiocarcinomas were observed (47% EH-CCA, 53% IH-CCA) with an age at the diagnosis higher for EH-CCA. Coexistence of cirrhosis or viral cirrhosis was more frequent in IH-CCA than EH-CCA. An incidental asymptomatic presentation occurred in 28% of IH-CCA vs 4% EH-CCA whilst, 74% EH-CCA vs 28% IH-CCA presented with jaundice. 91% of IH-CCA presented as a single intra-hepatic mass, whilst 50% of EH-CCA was peri-hilar. In the diagnostic work-up, 70% of all cholangiocarcinoma cases received at least 3 different imaging procedures. Tissue-proven diagnosis was obtained in 80% cholangiocarcinoma. Open surgery with curative intent was performed in 45% of IH-CCA and 29% EH-CCA. 18% IH-CCA vs 4% EH-CCA did not received treatment.
  
  
  CONCLUSIONS
  In Italy IH-CCA is managed as frequently as EH-CCA. In comparison to EH-CCA, IH-CCA occurs at younger age and is more frequently associated with cirrhosis and with an incidental asymptomatic presentation. In contrast, most EH-CCAs are jaundiced at the diagnosis. Cholangiocarcinoma diagnostic management is cost- and time-consuming with curative surgical treatment applicable more frequently in IH-CCA.
authors.affiliation: Department of Clinical Medicine, Sapienza University of Rome, Italy. domenico.alvaro@uniroma1.it
authors.forename: Domenico
authors.initials: D
authors.lastname: Alvaro
authors.forename: Maria Consiglia
authors.initials: MC
authors.lastname: Bragazzi
authors.forename: Antonio
authors.initials: A
authors.lastname: Benedetti
authors.forename: Luca
authors.initials: L
authors.lastname: Fabris
authors.forename: Giammarco
authors.initials: G
authors.lastname: Fava
authors.forename: Pietro
authors.initials: P
authors.lastname: Invernizzi
authors.forename: Marco
authors.initials: M
authors.lastname: Marzioni
authors.forename: Gennaro
authors.initials: G
authors.lastname: Nuzzo
authors.forename: Mario
authors.initials: M
authors.lastname: Strazzabosco
authors.forename: Tommaso
authors.initials: T
authors.lastname: Stroffolini
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000368:Aged; D001650:Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D017734:Bile Ducts, Extrahepatic / Q000000981:diagnostic imaging / Q000473:pathology*; D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging / Q000473:pathology*; D018281:Cholangiocarcinoma / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D003625:Data Collection; D003967:Diarrhea / Q000209:etiology; D005221:Fatigue / Q000209:etiology; D005260:Female; D006526:Hepatitis C / Q000150:complications; D006801:Humans; D007558:Italy; D007565:Jaundice / Q000209:etiology; D008103:Liver Cirrhosis / Q000150:complications; D008297:Male; D008875:Middle Aged; D009325:Nausea / Q000209:etiology; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D014839:Vomiting / Q000209:etiology; D015431:Weight Loss