Mucin-Producing Bile Duct Tumor

ABSTRACT

BACKGROUNDS A mucin-producing bile duct tumor is a rare disease causing diffuse bile duct dilatation and intermittent obstructive jaundice due to mucin accumulation in the bile duct. A massive amount of mucin in the dilated bile duct is confirmed during surgery, endoscopic drainage, or percutaneous drainage. These tumors are usually slow-growing, well-differentiated papillary adenocarcinomas that shows intraductal spreading or an intraductal papillary growth pattern. PURPOSE: The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of mucin-producing bile duct tumors. METHODS: We treated 10 patients with mucin-producing bile duct tumors during the recent 5 years. Clinical features were reviewed, including symptoms and signs, radiologic characteristics, operative findings, and pathological characteristics. RESULTS: The main clinical symptoms were recurrent abdominal pain, fever, chill, and intermittent jaundice. The characteristic radiologic findings were marked dilatation of the bile ducts distal to the tumors on computed tomography (CT) and ultrasonography and multiple, large, amorphous filling defects on cholangiography. In three cases, percutaneous transhepatic cholangioscopy (PTCS) was useful not only in making a diagnosis but also in delineating the extents of the tumors. In seven of ten cases involved (70%), the tumors were located mainly in the left intrahepatic duct. Pathologically, six (60%) cases were differentiated papillary adenocarcinoma. CONCLUSIONS: A mucin-producing bile duct tumor should be suspected if the patient has diffuse bile duct dilatation without definite evidence of stones, and it can be confirmed by a large amount of mucin secretion. If this type of tumor is suspected, careful preoperative evaluation, including PTCS, should be considered for making an accurate preoperative diagnosis and for determining the extent of the tumor.