ArticleACG case reports journal2026
Intrahepatic Cholangiocarcinoma in Small-Duct Primary Sclerosing Cholangitis Associated With Crohn Disease: First Reported Case and Surveillance Implications.
Article in ACG case reports journal, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Small-duct primary sclerosing cholangitis (sd-PSC) is traditionally regarded as a milder variant of PSC with a lower risk of hepatobiliary malignancy. Its association with intrahepatic cholangiocarcinoma (iCCA) remains unexplored. A 61-year-old woman with newly diagnosed Crohn disease presented with weight loss, anemia, and a liver mass initially suspected as hepatocellular carcinoma; biopsy confirmed poorly differentiated iCCA (CK7+/CK19+/Arginase-). MRCP showed normal large ducts with focal intrahepatic dilatation; liver biopsy demonstrated periductal onion-skin fibrosis diagnostic of sd-PSC. This report documents the first instance of metastatic iCCA in sd-PSC-CD, challenging current hepato-biliary malignancy surveillance paradigms in patients with inflammatory bowel disease.
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