Resection of a granular cell tumor at the hepatic confluence. A precarious location for a benign tumor

Granular cell tumors are lesions that account for less than 10 per cent of benign biliary tumors and only 1 per cent occur in the biliary tract. Since the first description, 45 other cases have been described to which we add a case. In reviewing the literature, a striking predominance in black females is evident. The most frequent location of these tumors in the biliary tree appears to be the common bile duct (50%), followed by the cystic duct (37%), the hepatic ducts (15%), and the gallbladder (4%). Most patients have been treated with resection followed by restoration of biliary continuity using biliary enteric diversion. In the case presented, the tumor was located at the hepatic confluence with extension into the hepatic substance requiring segmental duct cholangiojejunostomy after resection. This resulted in fulminant sepsis and death despite adequate preoperative biliary decompression and the lack of signs or symptoms of infected bile. Alternative forms of management in the treatment of this benign condition at this location are discussed.