[Biliary mucinous cystadenocarcinoma of the liver]

Biliary mucinous cystadenocarcinoma is an extremely rare tumour. Less than 50 cases have been reported. It is usually a multilocular cystic tumour covered with mucous producing epithelium, with papillary excrescences containing mucinous mass arising from bile ducts. The size of the tumour varies from 3.5 to 25 cm in diameter. It is more frequent in women. The majority of patients belong to the middle age population. We present a 63-year-old man who had been suffering from an epigastric and right subcostal pain of unknown aetiology for over 35 years. During the last 10 years he suffered from multiple attacks of cholangitis with high temperature, rigor, chills, pain and obstructive jaundice. Five years ago he had the attack of pancreatitis with retroperitoneal fatty necrosis for which he was operated on in another institution and cholecystectomy and pancreatic necrectomy were carried out. The attacks of cholangitis continued they were more serious and more frequent until June 1987, when the "cyst" in the left lobe of the liver, dilated bile ducts and "polyps" in the common bile duct were diagnosed by ultrasonography. During the operation advanced biliary cirrhosis, portal hypertension, splenomegaly, very dilated common bile duct full of jelly and the "cyst" in the liver filled with jelly, were found. The removal of the jelly and choledochojejunostomy resulted in temporary relief. Two months later he was reoperated for recurrent obstructive jaundice during which left lobectomy, partial excision of the cyst and cystojejunostomy between the rest of the cyst and another Roux-en-Y jejunal limb, were carried out.(ABSTRACT TRUNCATED AT 250 WORDS)