Granular cell tumor of the common hepatic duct presenting as cholangiocarcinoma and acute acalculous cholecystitis.

Granular cell tumors (GCT) are rare benign tumors. Less than 1% of GCTs involve the extrahepatic biliary tree. Most researches favour a Schwann cell origin. Patient, caucasion, female, 31-year-old presented with 4 month history of painless jaundice and pruritus. US and CT revealed dilatation of intrahepatic biliary tree and surgery was performed. Firm tumor mass was found above the conjunction of cystic duct and common hepatic duct (CHD) that caused obstruction and gallblader empyema. The patient underwent radical surgical procedure because Klatskin tumor was clinically suspected. Patohystology and immunohistochemistry confirmed granular cell tumor. Eight years after surgery the patient is wellbeing without symptoms. To our knowledge 69 cases of GCT of the extrahepatic biliary tree have been reported and none of the acute acalculous cholecystitis case acompanied by GCT of CHD. Granular cell tumors are rarely diagnosed preoperatively. Wide resection offers the best chance for cure.