Purpose of review: Cholangiocarcinoma is a devastating, unpredictable complication of large duct primary sclerosing cholangitis (PSC), which occurs in 5-15% of patients. The aim of this review is to discuss whether dominant strictures (DS) occurring in the larger bile ducts in PSC are a risk factor for the development of cholangiocarcinoma.
Recent findings: The development of DS is related to specific genetic polymorphisms affecting the innate immune system and the microbiome. In a recent study, the mean survival of PSC patients with DS was much worse (13.7 years) than for those without a DS (23 years). Survival difference was related to a 26% risk of cholangiocarcinoma, which developed only in those with DS. Half of the patients with cholangiocarcinoma presented within 4 months of the diagnosis of PSC. In another study, the risk of developing cholangiocarcinoma was directly related to the presence of underlying IBD, although this remains controversial. Efforts are being made towards surveying for cholangiocarcinoma including magnetic resonance imaging, endoscopic surveillance and serum tumour markers, but so far, an effective surveillance strategy has not been identified. DS should be treated endoscopically in the setting of symptoms, and there is limited evidence to suggest this may impact protectively on progression to cholangiocarcinoma.
Summary: It is established that the presence of symptomatic DS occurring in the larger bile ducts in PSC can be the first presentation of cholangiocarcinoma. There is an increasing body of evidence that even when proven to be benign, dominant biliary strictures predispose to the future development of cholangiocarcinoma. Regular surveillance should be targeted at this selected high-risk group of PSC patients.
Keywords: Cholangiocarcinoma; Cholangioscopy; Dominant stricture; Inflammatory bowel disease; Primary sclerosing cholangitis.
Conflict of interest statement
Conflict of Interest
Roger W. Chapman and Kate D. Williamson each declare no potential conflicts of interest.
Human and Animal Rights and Informed Consent
This article contains no studies with human or animal subjects performed by any of the authors.
Cholangiocarcinoma and Dominant Strictures in Patients With Primary Sclerosing Cholangitis: A 25-year Single-Centre ExperienceMH Chapman et al. Eur J Gastroenterol Hepatol 24 (9), 1051-8. PMID 22653260.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 ass …
Carcinoembryonic Antigen Level in Primary Sclerosing Cholangitis Is Not Influenced by Dominant Strictures or Bacterial CholangitisA Wannhoff et al. Dig Dis Sci 62 (2), 510-516. PMID 27943017.Serum CEA level is not influenced by the presence of DS or SBC and might therefore serve as a favorable parameter for improving cancer screening in PSC patients.
Endoscopic Assessment of Primary Sclerosing CholangitisB De Vries et al. Minerva Gastroenterol Dietol 62 (1), 49-62. PMID 26446685. - ReviewPrimary sclerosing cholangitis (PSC) is a rare chronic liver disease of unknown etiology for which the only known curative treatment is liver transplantation. The disease …
Endoscopic Retrograde Cholangiopancreatography for Primary Sclerosing CholangitisN Thosani et al. Clin Liver Dis 18 (4), 899-911. PMID 25438290. - ReviewAlthough there are no randomized, controlled trials evaluating the efficacy of endoscopic retrograde cholangiography (ERC) in primary sclerosing cholangitis (PSC) patient …
Primary Sclerosing Cholangitis and CholangiocarcinomaKN Lazaridis et al. Semin Liver Dis 26 (1), 42-51. PMID 16496232. - ReviewPrimary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by fibrosing inflammatory damage of the biliary tree, is a risk factor for cholangiocarcin …
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