title: Characterization and clinical course of hepatobiliary carcinoma in patients with primary sclerosing cholangitis.
abstract: OBJECTIVE
  Primary sclerosing cholangitis (PSC) confers a high risk of development of hepatobiliary carcinoma (HBC). The aim of the study was to identify indicators and risk factors for developing HBC in PSC patients.
  
  
  METHODS
  Thirty-nine PSC patients with HBC at time of HBC diagnosis were compared with 101 PSC patients without HBC at time of acceptance for liver transplantation.
  
  
  RESULTS
  Eighteen of these patients (46.2%) developed HBC within one year after diagnosis of PSC. In PSC patients with HBC male gender, nicotine abuse, long duration of inflammatory bowl disease (IBD), clinical symptoms, elevation of CA 19-9, as well as dominant bile duct stenosis were significantly more frequent (p<0.05) compared with the PSC control group. A cross-validated sensitivity and specificity of 85% and 97%, respectively, for the detection of HBC was obtained using the following parameters: weight loss, elevation of CA 19-9 >or= 200 kU/l, and dominant bile duct stenosis.
  
  
  CONCLUSIONS
  HBC is not necessarily a late complication of end-stage PSC. A long history of IBD, male gender, and nicotine abuse are risk factors for the development of HBC. In particular, CA 19-9, body-weight and dominant bile duct stenosis are valuable indicators in detecting HBC in PSC patients.
authors.affiliation: Department of Gastroenterology, Hepatology and Endocrinology, Medical School of Hannover, Germany.
authors.forename: Jens J W
authors.initials: JJ
authors.lastname: Tischendorf
authors.forename: Peter N
authors.initials: PN
authors.lastname: Meier
authors.forename: Christian P
authors.initials: CP
authors.lastname: Strassburg
authors.forename: Jürgen
authors.initials: J
authors.lastname: Klempnauer
authors.forename: Hartmut
authors.initials: H
authors.lastname: Hecker
authors.forename: Michael P
authors.initials: MP
authors.lastname: Manns
authors.forename: Martin
authors.initials: M
authors.lastname: Krüger
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001660:Biliary Tract Diseases / Q000150:complications; D001661:Biliary Tract Neoplasms / Q000209:etiology*; D001835:Body Weight; D018395:CA-19-9 Antigen / Q000032:analysis; D015209:Cholangitis, Sclerosing / Q000150:complications*; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications*; D008113:Liver Neoplasms / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012737:Sex Factors; D012907:Smoking / Q000009:adverse effects
chemical_list: D018395:CA-19-9 Antigen