biliary cystadenocarcinoma is a very rare cystic tumor and constitutes less than 5% of intrahepatic cysts of biliary origin . along with the recent advances in diagnostic imaging techniques , we report a case of biliary cystadenocarcinoma in a 63-year - old man with a review of the literature . a 63-year - old man was readmitted to our hospital for further evaulation of hepatic cystic lesion . one year prior to admission , he had visited a local hospital due to pain in the right upper quadrant of the abdomen and fever . abdominal sonographic finding showed about a 10cm - sized cystic nature space occupying lesion in the right lobe of the liver ( fig . 1 ) and the laboratory tests were as follows : leukocyte , 12,900/mm ; hemoglobin , 12.9g / dl ; total bilirubin , 4.6mg / dl ; ast , 39iu / l ; alt , 74iu / l ; alkaline phosphatase , 590iu / l ; ggt , 90iu / l . after discharge , he did not follow up with any specific problem for about 1 year . upon readmission , his temperature was 37.0c , pulse 80 per minute , respiration 20 per minute . there was no pathologic lesion in his eyes , ears , nasal or oral mucosa . on auscultation of chest , breathing sounds were normal , and the heart sound was regular without murmur . the results of a routine examination showed a hemoglobin of 13.3g / dl , hematocrit of 39% , a leukocyte count of 11,600/mm ( neutrophil 67% , lymphocyte 27% , monocyte 6% ) and a platelet count of 273,000/mm . liver function test showed as follows : total bilirubin , 0.9mg / dl ; direct bilirubin , 0.6mg / dl ; ast , 29iu / l ; alt , 13iu / l ; alkaline phosphatase , 70iu / l ; ggt , 38iu / l . all tumor markers were within normal limits ; afp , 3.1ng / ml ; cea , 0.4ng / ml ; ca 19 - 9 , 8.96u / ml and ca 125 , 40.1u / ml . chest and abdominal x - ray revealed no abnormality . abdominal ct demonstrated huge cystic lesions measuring 1010 cm in right liver and having focal irregular wall thickening with internal papillary projections ( fig . endoscopic retrograde cholangiography ( ercp ) revealed mild dilated common bile duct and some filling defect in right intrahepatic duct . the cystic lesion was connected with right intrahepatic duct and some filling defect was seen in the cystic lesion ( fig . celiac angiography showed a large cavitary lesion which is supplied by hepatic artery ( fig . on laparotomy , a huge hepatic mass was occupying in segment v and the cystic mass contained a portion of the right hepatic duct . the aspirated material from the cyst was found to be the same as the mucinous material drained endoscopically . the surgical specimen contained a grayish - brown colored , well encapsulated unilocular cyst measuring 10.08.05.0 cm ( fig . the internal cavity was filled with multiple papillary tumors and abundant mucinous fluid ( fig . histologic examination showed papillary proliferation of atypical cuboidal epithelium with mucin production in the cystic area . the patient is doing well 2 year and 2 months after the operation and without a sign of recurrence . biliary cystadenocarcinoma is a very rare cystic tumor and it has been reported to arise from congenital liver cysts , bile ducts , biliary cystadenoma , fibropolycystic disease and hepatoduodenal ligament . the tumor size ranges from 3 cm to 26 cm in the largest diameter and , even though some patients are asymptomatic , most of them are large in size accounting for the symptoms and sign , such as abdominal distention , nausea , palpable abdominal masses and abdominal local pain . however , with recent progress in imaging diagnosis , cystadenocarcinoma has been diagnosed with relative ease and the rapid enlargement of the tumor may be a possible sign of malignancy , but the clinical diagnosis is difficult during the preopeative course . the typical ultrasonographic appearance is that of a globular or ovoid thick - walled cystic mass . fluid or low level echoes may be seen within the cyst , a sonographic appearance similar to that of pancreatic or ovarian cystadenomas or cystadenocarcinomas . ct may demonstrate a multilobulated cystic mass that contains septations , papillary projections and , sometimes mural nodules . ercp and percutaneous transhepatic cholangiography demonstrate whether there are an intraluminal obstructing lesion and direct communication between the cyst lumen and the biliary tree , or not . in our case , ercp showed that the cystic lesion was connected with the right hepatic duct and it suggests that the cyst has arisen in the bile duct . angiography demonstrates a hypovascular mass in most instances or , sometimes , a small vascular blush along the periphery of the tumor as in our case . grossly , the tumor is usually large - size and consists of a solitary , well encapsulated , fluid - containin multilocular cyst . wee et al reported a case of biliary adenocarcinoma which was diagnosed preoperatively based on the fine needle aspiration cytologic findings and the imaging appearances . but , the results are usually negative and the chance of peritoneal seeding of malignant cells is present with such a procedure . histopathologically , the epithelium lining the cystic spaces is mostly nonciliated columnar to cuboidal mucin secreting epithelium and portions of transition from cystadenoma to cystadenocarcinoma tumor cells may be seen . most epithelial tumor cells are positive on immunohistochemical staining with antibodies to cytokeratin , epithelial membrane antigen and carcinoembrionic antigen . the prognosis is comparatively good with complete resection and generally seems to be much better than that for intrahepatic bile duct carcinoma . recently , devaney et al recognized that there was a close relation between morphological findings and prognosis . one is seen in female patients and arise from a preexisting biliary cystadenoma , usually accompanied by ovarian stroma , and follows an indolent course . the other is found in the male , not associated with a preexisting biliary cystadenoma with ovarian stroma , is more aggressive and leads to death in over half the patients . in conclusion , we report here a rare case of biliary cystadenocarcinoma in a 63-year - old man who was treated for liver abscess , although our case was unilocular , which is not typical .
biliary cystadenocarcinoma is a very rare cystic tumor that arises in the liver or , less frequently , in the extrahepatic biliary system . 
 it has been shown to arise in congenital liver cysts , bile ducts , biliary cystadenoma , in the context of fibro - polycystic disease and in the hepatoduodenal ligament . 
 common presenting symptoms include an abdominal mass , local pain , nausea , jaundice , fever or occasional ascites . some patients are asymptomatic , the lesion being an incidental finding at autopsy or surgery . 
 approximately 50 cases have been reported in the literature.we report a case of biliary cystadenocarcinoma in a 63-year - old man with a review of the literature .