Table 1. Malignancy screening in primary sclerosing cholangitis.

Cholangiocarcinoma	Investigation for CCA should be pursued with clinical suspicion based on:38 Altered liver biochemical parameters Development of dominant biliary stricture Decline in constitutional performance status Clinical symptoms such as jaundice, abdominal pain, or weight loss Suggested screening approach includes:38 MR imaging (MRI/MRCP) of liver Measurement of serum CA 19-9 ERCP for investigation of dominant stricture or elevated CA 19-9; biliary biopsy and brush cytology with molecular testing by FISH analysis where available
Colorectal Cancer	Colonoscopy with biopsies at initial PSC diagnosis and thereafter as follows:11,38 Surveillance colonoscopy with biopsies every 1 year for PSC patients who have IBD or are post-liver transplantation11,191 Surveillance colonoscopy with biopsies every 5 years in PSC patients without IBD192 UDCA is currently not recommended for chemoprevention in PSC-UC38
Gallbladder Cancer	Abdominal ultrasound yearly11,38 Detection of mass lesion(s) in the gallbladder should prompt cholecystectomy in appropriate surgical candidates11,38
Hepatocellular Carcinoma	Abdominal ultrasound every 6 months193 Measurement of serum alpha-fetoprotein can be considered

Abbreviations: CCA (cholangiocarcinoma); MRI (magnetic resonance imaging); MRCP (magnetic resonance cholangiopancreatography); CA (carbohydrate antigen); ERCP (endoscopic retrograde cholangiopancreatography); FISH (fluorescence in situ hybridization); PSC (primary sclerosing cholangitis); IBD (inflammatory bowel disease); UDCA (ursodeoxycholic acid); UC (ulcerative colitis).