Curbside Consultation in GI Cancer for the Gastroenterologist: 49 Clinical Questions

Douglas G. Adler, MD

Question 24: A 24-Year-Old Woman Is Found To Have Primary Sclerosing Cholangitis. What Is Her Risk Of Developing Cholangiocarcinoma? (continued)

James D. Morris, MD; Virendra Joshi, MD, AGAF

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease marked by fibrosis and inflammation of the intrahepatic and extrahepatic bile ducts leading to biliary cirrhosis (Figure 24-1). Large proportions (60% to 80%) of patients have associated inflammatory bowel disease (IBD), most often ulcerative colitis. Cholangiocarcinoma (CCA) represents the most dreaded complication of PSC. CCA can occur at any location in the biliary tree and at any stage of PSC. The male-to-female ratio of CCA is 1.5:1, which parallels that of PSC patients overall. The overall risk for the…