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.
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.
Alvaro
Domenico
D
Department of Clinical Medicine, Sapienza University of Rome, Italy. domenico.alvaro@uniroma1.it
Bragazzi
Maria Consiglia
MC
Benedetti
Antonio
A
Fabris
Luca
L
Fava
Giammarco
G
Invernizzi
Pietro
P
Marzioni
Marco
M
Nuzzo
Gennaro
G
Strazzabosco
Mario
M
Stroffolini
Tommaso
T
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