Abstract
A 47-year-old woman was admitted to our hospital with a history of upper abdominal pain. She gave a history of having undergone extrahepatic bile duct excision for congenital biliary dilatation when she was 18 years old. Imaging findings showed a carcinoma of the head of the pancreas invading the superior mesenteric vein, duodenum and reconstructed jejunal loop. Subtotal stomach-preserving pancreatoduodenectomy was performed, with resection of the portal vein. Histopathological examination of the resected specimen revealed a well-differentiated tubular adenocarcinoma of the head of the pancreas invading the surrounding vessels and organs, consistent with the preoperative imaging findings. However, there was no lymph node metastasis and no remnant intrapancreatic bile duct. As there are a few case reports of carcinoma of the pancreas associated with pancreaticobiliary maljunction, the risk of carcinogenesis of the pancreatic duct is a cause for concern in young patients undergoing primary excision for congenital biliary dilatation and these patients need to be followed up carefully.
