title: Cholangiocarcinoma complicating primary sclerosing cholangitis: a 24-year experience.
abstract: OBJECTIVE
  To report the prevalence and outcome of cholangiocarcinoma arising in primary sclerosing cholangitis for a British tertiary referral centre.
  
  
  METHODS
  All patients diagnosed with primary sclerosing cholangitis and concurrent cholangiocarcinoma were identified from a prospectively maintained departmental database, and the mode of presentation, management and outcome were determined.
  
  
  RESULTS
  Of 370 patients with primary sclerosing cholangitis, 48 patients (13%) were diagnosed with a cholangiocarcinoma within a median time of 0.51 months (range: 0-73.12) from presentation to the unit. Mode of presentation included: inoperable tumours (n = 14); incidental findings in transplant hepatectomy specimens (n = 13); primary sclerosing cholangitis follow-up (n = 9); transplant work-up (n = 5); transplant waiting list (n = 5); suspected tumour confirmed at transplant (n = 1), and incidental finding at cholecystectomy (n = 1). The diagnosis was confirmed by: radiology-guided biopsy (n = 27); MRI (n = 3); CT (n = 2); laparoscopy or laparotomy (n = 2), and frozen section at transplant (n = 1). Management consisted of: transplantation (n = 14, including 1 abandoned); hepatic resection (n = 8), and palliation through stenting (n = 26). The overall median survival of the cohort was 4.9 months (range: 0.09-104.5). Median survival ranged from 2.6 months (range: 0.09-35.3) for palliation to 7.6 months (range: 0.6-99.6) for transplantation and 52.8 months (range: 3.7-104.5) for resection. There was no difference in survival between the transplant and resection groups (p = 0.14).
  
  
  CONCLUSIONS
  Cholangiocarcinoma is a common finding in primary sclerosing cholangitis and regular screening of this cohort of patients at referring centres is advocated to detect early tumours, as surgical treatment at an early stage offers significantly better outcomes for this cohort of patients.
authors.affiliation: Department of Hepatobiliary and Liver Transplant Surgery, Queen Elizabeth Hospital, Birmingham, UK. garethmorrisstiff@hotmail.com
authors.forename: G
authors.initials: G
authors.lastname: Morris-Stiff
authors.forename: C
authors.initials: C
authors.lastname: Bhati
authors.forename: S
authors.initials: S
authors.lastname: Olliff
authors.forename: S
authors.initials: S
authors.lastname: Hübscher
authors.forename: B
authors.initials: B
authors.lastname: Gunson
authors.forename: D
authors.initials: D
authors.lastname: Mayer
authors.forename: D
authors.initials: D
authors.lastname: Mirza
authors.forename: J
authors.initials: J
authors.lastname: Buckels
authors.forename: S R
authors.initials: SR
authors.lastname: Bramhall
mesh_terms: D000328:Adult; D000368:Aged; D001650:Bile Duct Neoplasms / Q000150:complications / Q000401:mortality / Q000601:surgery; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000150:complications* / Q000401:mortality / Q000601:surgery; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000401:mortality; D005260:Female; D006498:Hepatectomy; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D015607:Stents