Granular cell tumor of the biliary system

Granular cell tumors (GCT) are rare benign neoplasms of Schwann cell origin which have been found in virtually every location in the body. Their location in the biliary system is unusual, and typically occurs in black females during the fourth decade of life. Forty-eight cases have been reported since 1952. We present two patients treated for obstructive jaundice caused by GCT of the extrahepatic biliary tree. The literature on biliary GCT is reviewed and their management is outlined. Local excision with Roux-y-hepaticojejunostomy was performed in one patient with GCT obstructing the common bile duct at the level of the cystic duct. Pancreaticoduodenectomy was performed on one patient for GCT of the common bile duct involving the ampulla and adjacent pancreas. GCT of the extrahepatic biliary system are rare but should be considered in black females who present with obstructive jaundice in the fourth decade of life. Local excision with cholecystectomy and reconstruction of the biliary remnant is indicated. Primary biliary anastomosis is desired but choledochojejunostomy or hepaticojejunostomy may be necessary. Rarely, pancreaticoduodenectomy may be indicated in GCT of the distal common bile duct.