title: Low risk of hepatocellular carcinoma in patients with primary sclerosing cholangitis with cirrhosis.
abstract: OBJECTIVE
  Primary sclerosing cholangitis (PSC) is associated with an increased risk of hepatobiliary malignancies. However, little is known about the incidence of hepatocellular carcinoma (HCC) among patients with PSC; current recommendations on screening these patients for HCC are conflicting. We investigated the risk of HCC in patients with PSC with cirrhosis.
  
  
  METHODS
  We performed a retrospective study of patients with well-defined PSC from 2 large-volume tertiary care centers in Germany; data were collected from periods of up to 33 years. Liver cirrhosis was based on histology results or the presence of ascites, esophageal varices, or transient elastography values greater than 14 kPa. Statistical analysis was performed using the Kaplan-Meier method and the Cox proportional hazard model. Data from 509 patients (67% male), with a total of 4202 patients-years, were included in the final analysis.
  
  
  RESULTS
  We identified 119 patients with cirrhosis. During 292 patient-years, none of these patients developed HCC. Most HCCs were identified incidentally at the time of liver transplantation. We therefore reviewed data on liver explants from 140 patients who underwent transplantation; none were found to contain HCC. In contrast to the low numbers of HCCs among patients with PSC, 35 patients developed cholangiocarcinoma, 3 patients developed gallbladder cancer, and 9 patients developed colorectal cancer.
  
  
  CONCLUSIONS
  Based on a retrospective analysis of more than 500 patients with PSC, we confirm their high risk for hepatobiliary malignancies. However, the risk of HCC, even among patients with cirrhosis, seems to be low--regular HCC surveillance may not be warranted.
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Roman
authors: R
authors: Zenouzi
authors: Department of Internal Medicine 1, University of Bonn, Bonn, Germany; Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
authors: Tobias J
authors: TJ
authors: Weismüller
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Peter
authors: P
authors: Hübener
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Kornelius
authors: K
authors: Schulze
authors: Biostatistics Department, Rouen University Hospital-Charles Nicolle, Rouen, France.
authors: Michael
authors: M
authors: Bubenheim
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Nadine
authors: N
authors: Pannicke
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Christina
authors: C
authors: Weiler-Normann
authors: Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
authors: Henrike
authors: H
authors: Lenzen
authors: Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
authors: Michael P
authors: MP
authors: Manns
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Ansgar W
authors: AW
authors: Lohse
authors: First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address: cschramm@uke.de.
authors: Christoph
authors: C
authors: Schramm
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D056808:Biostatistics; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000150:complications*; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008103:Liver Cirrhosis / Q000150:complications*; D008113:Liver Neoplasms / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D018570:Risk Assessment; D062606:Tertiary Care Centers; D055815:Young Adult
keywords: Cholangiocarcinoma; Liver Cancer; Population; Screening