A Case of Agenesis of the Pancreatic Body and Tail with the Lower Bile Duct Carcinoma

Abstract

We report a very rare case of agenesis of the pancreatic body and tail with lower bile duct cancer. A 47-year-old woman followed up for diabetes mellitus was found to have liver function disorder in blood chemistry tests done due to general fatigue. Abdominal ultrasonography (US) showed a highly echoic 14 mm tumor in the lower bile duct. Endoscopic retrograde pancreatography (ERCP) showed a defect in the lower bile duct and a short main pancreatic duct in the pancreatic head, but the accessory pancreatic duct and minor papilla were absent. Plain computed tomography (CT) showed no tissue of the pancreatic body and tail. A biopsy specimen in percutaneous transhepatic cholangioscope showed adenocarcinoma, so we conducted pyloruspreserving total pancreatectomy based on a diagnosis of lower bile duct cancer with agenesis of the pancreatic body and tail. On laparotomy, the pancreatic body and tail could not be found, and the splenic vein was completely exposed. Histopathological findings showed well-differentiated tubular adenocarcinoma. Agenesis of the pancreatic body and tail is rare, especially in bile duct cancer, with our case being only the fifth reported in the Japanese literature.