Cancer surveillance in patients with primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a chronic fibroinflammatory syndrome involving the biliary tract, often accompanied by inflammatory bowel disease. This syndrome is a prototype disease linking chronic inflammation to carcinogenesis. Indeed, PSC is associated with an increased risk of cholangiocarcinoma, gallbladder cancer, hepatocellular carcinoma, and colorectal cancer. Herein, we review the risk for these malignancies in PSC and discuss rational cancer surveillance strategies for these patients. Where evidence is limited, we suggest a pragmatic approach. In this regard we recommend interval screening for cholangiocarcinoma with non-invasive imaging modalities and serum carbohydrate antigen 19-9 determinations annually. These imaging studies also serve to screen for gallbladder cancer and hepatocellular carcinoma. Screening for colorectal cancer is more firmly established in PSC patients with inflammatory bowel disease and includes colonoscopy at the time of PSC diagnosis and, thereafter, at 1-2 year intervals. We also highlight areas where more information is required such as management of biliary tract dysplasia and cancer chemoprevention in PSC.

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

“…PSC is a chronic cholestatic disorder with significantly increased risk of cholangiocarcinoma (CCA) that demands constant vigilance [11]. The heterogeneous presentation characterizing PSC involves at least four variants: "Classical" PSC (involving the intrahepatic or extrahepatic biliary tree or both), small-duct PSC, PSC/autoimmune hepatitis (AIH) overlap syndrome, and the IgG4 associated cholangitis (IgG4-SC) or immune associated cholangiopathy (IAC) [12].…”

Emerging therapies for the treatment of cholangiocarcinoma

“…In the Western world, primary sclerosing cholangitis (PSC) and fatty liver disease resulting in chronic inflammation of the biliary tree represent the most common predisposing conditions for CCA [8,9]. Other proposed risk factors include alcohol consumption [10], diabetes mellitus [10,11], opisthorchiasis (hepatobiliary flukes) [12,13], choledochal cysts [14], chronic hepatitis B and C [10,15], obesity [10,15,16], cirrhosis [10], hepatolithiasis [17], and chemical carcinogens [5].…”

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

“…In regard to CC, screening strategies employing imaging techniques, preferentially with MRI, but also ultrasound and computed tomography, in association with tumor biomarkers have been suggested strategies (241,244) , but their accuracy in detecting CC was reported to be low (244,245) . The most commonly employing biomarker for CC in PSC is CA19-9.…”

“…Values higher than 20 U/ml, when employed in conjunction to MRCP, can increase their specificity to levels near 100%, but with lower sensitivity (less than 40%). In addition, CA19-9 levels equal or higher than 129 U/ml have also been shown to increase specificity, but also with a significant decrease in sensitivity (244,245) . Higher levels of CA19-9 were also associated with advanced tumors, usually with adverse outcomes (241,245,246) .…”

“…Higher levels of CA19-9 were also associated with advanced tumors, usually with adverse outcomes (241,245,246) . Endoscopic retrograde colangiography with brushing citology is a method currently used for diagnostic confirmation of CC in PSC subjects with dominant strictures and/or with abnormal levels of CA19-9, but not for tumor screening due to its association with pottentialy serious adverse events (241,244,247) . The adittion of fluorescent in situ hybridization (FISH) of cytologic samples, disclosing polysomy in at least five biliary cless obtained by endoscopic brushing, was shown to enhance diagnostic accuracy for CC in PSC (68,75) .…”