Cholangiocarcinoma and Dominant Strictures in Patients With Primary Sclerosing Cholangitis: A 25-year Single-Centre Experience

Eur J Gastroenterol Hepatol. 2012 Sep;24(9):1051-8. doi: 10.1097/MEG.0b013e3283554bbf.


Background: Dominant biliary strictures occur commonly in patients with primary sclerosing cholangitis (PSC), who have a high risk of developing cholangiocarcinoma (CC). The natural history and optimal management of dominant strictures remain unclear, with some reports suggesting that endoscopic interventions improve outcome.

Methods: We describe a 25-year experience in patients with PSC-related dominant strictures at a single tertiary referral centre.

Results: A total of 128 patients with PSC (64% men, mean age at referral 49 years) were followed for a mean of 9.8 years. Eighty patients (62.5%) with dominant biliary strictures had a median of 3 (range 0-34) interventions, compared with 0 (0-7) in the 48 patients without dominant strictures (P<0.001). Endoscopic interventions included the following: (i) stenting alone (46%), (ii) dilatation alone (20%), (iii) dilatation and stenting (17%) and (iv) none or failed intervention (17%, of whom most required percutaneous transhepatic drainage). The major complication rate for endoscopic retrograde cholangiopancreatography was low (1%). The mean survival of those with dominant strictures (13.7 years) was worse than that for those without dominant strictures (23 years), with much of the survival difference related to a 26% risk of CC developing only in those with dominant strictures. Half of those with CC presented within 4 months of the diagnosis of PSC, highlighting the importance of a thorough evaluation of new dominant strictures.

Conclusion: Repeated endoscopic therapy in PSC patients is safe, but the prognosis remains worse in the subgroup with dominant strictures. In our series, dominant strictures were associated with a high risk of developing CC.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Bile Duct Neoplasms / surgery*
  • Bile Ducts, Intrahepatic / surgery*
  • Cholangiocarcinoma / surgery*
  • Cholangiopancreatography, Endoscopic Retrograde / methods
  • Cholangitis, Sclerosing / surgery*
  • Constriction, Pathologic / surgery
  • Female
  • Humans
  • Male
  • Middle Aged
  • Postoperative Complications / epidemiology
  • Prognosis
  • Stents
  • Tertiary Care Centers
  • Treatment Outcome