Abstract
We encountered a rare case of primary sclerosing cholangitis (PSC) localized in the intrahepatic bile duct (IHBD)(B7). A 71-year-old man was fond to have an abnormal mucosa in the stomach at a medical examination visited our hospital and was diagnosed with gastric cancer by an upper GI seies. At the same time, ultrasonography and a CT scan revealed dilation of the IHBD (B7). Endoscopic retrograde cholangiography did not indicate IHBD (B7) and PTC revealed dilatation and stenosis of IHBD (B7). Distal partial gastrectomy and posterior segmentectomy of the liver were performed with the diagnosis of gastric cancer and a suspicion of hepatolithiasis or cholangiocelluler carcinoma. The surgical specimen showed dilatation and stenosis of IHBD (B7), and no tumor formation or stone. Histological examination revealed periductal fibrosis with infiltration of inflammatory cells. We made the final diagnosis of intrahepatic localized PSC. To the best of our knowledge, this is the first of an intrahepatic localized PSC in our country. Surgical resection should be performed for the localized type of PSC, because of progress of this disease and its rare combination with cholangiocellular carcinoma.