Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibro-obliterative inflammation of the hepatic bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and in some cases, cholangiocarcinoma (CCA). The development of CCA in PSC is unpredictable, its surveillance and diagnosis complex, and its treatment options limited unless detected early. Herein we provide a focused review of the current literature regarding CCA surveillance in patients with PSC and discuss the diagnostic and management challenges that exist. Where evidence is limited, we present our perspective and approach as well as directions for future research.
Keywords: Neoplasm; bile duct diseases; bile duct neoplasms; biological tumor marker; cholangiography; cytological techniques; early detection of cancer; fluorescence in situ hybridization; imaging; magnetic resonance imaging; mass screening; ultrasonography.
Conflict of interest statement
DISCLOSURES, CONFLICTS OF INTEREST None.
Screening and Diagnosis of Cholangiocarcinoma in Patients With Primary Sclerosing CholangitisAB Jesudian et al. Rev Gastroenterol Disord 9 (2), E41-7. PMID 19668124. - ReviewPrimary sclerosing cholangitis (PSC) is an idiopathic cholestatic liver disease which predisposes to the development of cholangiocarcinoma (CCA). Detection of CCA in PSC …
Primary Sclerosing Cholangitis: A Review and UpdateJH Tabibian et al. Liver Res 1 (4), 221-230. PMID 29977644.Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and …
Precancerous Bile Duct Pathology in End-Stage Primary Sclerosing Cholangitis, With and Without CholangiocarcinomaJT Lewis et al. Am J Surg Pathol 34 (1), 27-34. PMID 19898228.Patients with primary sclerosing cholangitis (PSC) are at increased risk for developing cholangiocarcinoma (CCA). However, the morphology of precursor lesions and the pre …
Cholangiocarcinoma and Its Mimickers in Primary Sclerosing CholangitisJJ Lee et al. Abdom Radiol (NY) 42 (12), 2898-2908. PMID 28951947. - ReviewCholangiocarcinoma (CCA) is the most common malignancy in primary sclerosing cholangitis (PSC). Approximately half of CCA are diagnosed within two years of initial diagno …
The Challenges in Primary Sclerosing Cholangitis--Aetiopathogenesis, Autoimmunity, Management and MalignancyTJ Weismüller et al. J Hepatol 48 Suppl 1, S38-57. PMID 18304683. - ReviewPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by progressive inflammation and fibrosis of the bile ducts, resulting in biliar …