title: Clinical presentation, diagnosis, and survival in cholangiocarcinoma: A prospective study.
abstract: OBJECTIVE
  The diagnosis of cholangiocarcinoma (CCA) is difficult. The present study aimed to assess the clinical features, diagnosis, and survival in CCA.
  
  
  METHODS
  This is a prospective study on 46 patients with CCA who underwent endoscopic retrograde cholangiopancreatography (ERCP) or surgical resection and 20 controls with a clinical and ERCP suspicion for CCA in whom surgical biopsy and/or 4-year follow-up showed a benign biliary stricture.
  
  
  RESULTS
  The median age at presentation was 71years (range 44-88). Thirty-four patients (73.9%) presented with painless jaundice. Median CA 19-9 value was 188IU/L (range 1-49,138), with a level of <100IU/L in 13 patients (28%). Total bilirubin was 11.9 (0.6-36.3)mg/dL. The tumour was intrahepatic in 3 (6.5%), hilar (Klatskin) in 25 (54.3%), and located in the lower third of the bile duct in 18 (39.1%) patients. The diagnosis was confirmed by positive cytology in 10 (21.7%), biopsy in 20 (43.5%), cholangioscopy in five (10.9%), and imaging and clinical grounds in 11 (23.9%) patients. Cytology was feasible in 36 patients; it was positive in 10 and "highly indicative" in two patients (33.3% sensitivity). Twenty-two patients (47.8%) were treated by surgical resection, and the rest were offered palliative biliary drainage. Mean estimated survival for the entire group of CCA patients was 21.5±3.3months. Survival was slightly longer in patients who underwent surgical resection than those who had palliative treatment; the estimated mean survival rates were 26.2±4.2 vs. 17.1±3.3months, respectively, but the difference was not statistically significant (p=0.115).
  
  
  CONCLUSIONS
  The diagnosis of CCA is difficult and often delayed. The outcome is generally poor.
authors.affiliation: 2nd Department of Internal Medicine, "Hippokration" Hospital, Medical School, National and Kapodistrian University of Athens, Athens 11527, Greece.
authors.forename: Larisa
authors.initials: L
authors.lastname: Vasilieva
authors.affiliation: Hematology Laboratory, "G. Gennimatas" General Hospital, Athens 11527, Greece.
authors.forename: Stefanos I
authors.initials: SI
authors.lastname: Papadhimitriou
authors.affiliation: 2nd Department of Internal Medicine, "Hippokration" Hospital, Medical School, National and Kapodistrian University of Athens, Athens 11527, Greece.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Alexopoulou
authors.affiliation: 2nd Department of Internal Medicine, "Hippokration" Hospital, Medical School, National and Kapodistrian University of Athens, Athens 11527, Greece.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Kostopoulos
authors.affiliation: Endoscopic Surgery Unit, Hippokration Hospital, Athens 11527, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Papiris
authors.affiliation: Hematology Laboratory, "G. Gennimatas" General Hospital, Athens 11527, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Pavlidis
authors.affiliation: Gastroenterology Unit, "Agios Savvas" Cancer Hospital, Athens 11521, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Xinopoulos
authors.affiliation: Endoscopic Surgery Unit, Hippokration Hospital, Athens 11527, Greece.
authors.forename: Andreas
authors.initials: A
authors.lastname: Romanos
authors.affiliation: 2nd Department of Internal Medicine, "Hippokration" Hospital, Medical School, National and Kapodistrian University of Athens, Athens 11527, Greece. Electronic address: spdour@med.uoa.gr.
authors.forename: Spyridon P
authors.initials: SP
authors.lastname: Dourakis
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000469:Alkaline Phosphatase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D001650:Bile Duct Neoplasms / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D017734:Bile Ducts, Extrahepatic / Q000473:pathology*; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D001663:Bilirubin / Q000097:blood; D001706:Biopsy; D018395:CA-19-9 Antigen / Q000097:blood; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003251:Constriction, Pathologic / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology; D004322:Drainage; D005260:Female; D006801:Humans; D007565:Jaundice / Q000209:etiology; D018285:Klatskin Tumor / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D010166:Palliative Care; D011446:Prospective Studies; D015996:Survival Rate
chemical_list: D018395:CA-19-9 Antigen; D001219:Aspartate Aminotransferases; D000469:Alkaline Phosphatase; D001663:Bilirubin
keywords: Cholangiocarcinoma; Klatskin tumour; Mortality