Cholangiography of mucin-hypersecreting intrahepatic biliary neoplasms

Bile duct obstruction caused by profuse mucin secretion in a major bile duct is rare. Such cases are often incorrectly identified as choledocholithiasis before being histologically proven. The goal of this study was to analyze the cholangiograms of mucin-hypersecreting intrahepatic biliary neoplasms and to try to identify the characteristic cholangiographic features of this rare disease. A retrospective analysis of 20 cholangiograms (from 6 men and 14 women) of mucin-hypersecreting intrahepatic biliary neoplasms over a 13-year period was carried out. Sixty percent of the patients had associated biliary tract stones. A mucinous substance drained out during biliary catheterization (endoscopic retrograde cholangiography [ERC]; percutaneous transhepatic cholangiography [PTC] in 4 patients. The cholangiographic findings included: (1) The appearance of diffuse "ground-glass" filling defects of extrahepatic duct and non-visualization of the tumor-bearing intrahepatic duct in 9 patients (45%), (2) multiple amorphous filling defects of the extrahepatic duct and tumor-bearing intrahepatic duct in 5 patients (25%), (3) multiple filling defects of the extrahepatic duct and non-visualization of the tumor-bearing intrahepatic duct in 4 patients (20%), and (4) single amorphous filling defect of the extrahepatic duct in 2 patients (10%). Mucin-hypersecreting intrahepatic tumors with mucobilia should be highly suspected when the following cholangiographic characteristic are shown: (1) mucinous substance draining out during catheterization (ERC or PTC); (2) diffuse "ground-glass" appearance filling defects; and (3) multiple amorphous filling defects of the bile duct associated with a cystic mass density within the liver on sonography or computed tomographic scan.