Background/Aims: Mucin-hypersecreting bile duct tumor, which closely resembles intraductal papollary mucinous tumor (IPMT) of the pancreas, is rare and its clinical features are not well known. We report our experience of nine patients with the tumor and analyze the cases to elucidate its clinicopathologic characteristics. Methods: Between 1995 and 1998, nine consecutive patients (4 men and 5 women, mean age 54 years) who were diagnosed as a mucin-hypersecreting bile duct tumor were enrolled in this study. Results: Recent or previous attacks of biliary pain and acute cholangitis were elicited in most of the patients. A widely open ampulla of Vater with extrusion of mucin and an diffuse dilated intra-and extrahepatic bile duct with amorphous filling defects on cholangiogram were characteristic. On cholangioscopic examination, papillary mass or minute mucosal lesion was found in the dilated bile duct containing thick viscid mucin. Nine patients were referred to an operation and curative resection was performed in eight patients. Histologically, well-differentiated adenocarcinoma in the background of benign hyperplasia and adenoma was documented in all patients except one, who showed pure adenoma. Conclusion: Mucin-hypersecreting bile duct tumor may be characterized by striking homology with IPMT of the pancreas in clinical, radiological and pathological features.
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