A Case of Carcinoma Arising in the Remnant Intrapancreatic Bile Duct 13 Years after Primary Excision of a Choledochal Cyst without Pancreatobiliary Maljunction

Keinosuke Ishido, Yoshikazu Toyoki, Syoujiroukazunori Ikenaga, Shinji Tsutsumi, Shunji Narumi and Kenichi Hakamada

Department of Gastrointestinal Surgery, Hirosaki University Graduate School of Medicine

We report herein a case of carcinoma in the intrapancreatic bile duct developing 13 years after initial choledochal cyst surgery without pancreatobiliary maljunction. A 41-year-old woman admitted for epigastralgia and weight loss. Thirteen years earlier, she had undergone choledochal cyst surgery (Alonso-Lej type Ia without pancreatobiliary maljunction), which involved choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Upper gastrointestinal fiberscopy showed a severe pyloric stenosis and computed tomography showed a mass 25 mm in diameter at the head of the pancreas with severe duodenal bulb invasion. PET showed FDG accumulation at the pancreatic head, corresponding to the mass found in CT. Under a diagnosis of intrapancreatic remnant bile duct cancer, we conducted substomach-preserving pancreaticoduodenectomy. Histopathologic examination showed that tubular adenocarcinoma had developed in the intrapancreatic remnant bile duct with severe invasion to the duodenum and pancreas. Adjuvant chemotherapy with TS-1 (100 mg/body) was started 4 weeks postoperatively, and no evidence of recurrence or distant metastasis has been detected 6 months after the operation.

Key words
choledochal cyst, remnant bile duct, pancreatobiliary maljunction

Reprint requests
Keinosuke Ishido Department of Gastrointestinal Surgery, Hirosaki University Graduate School of Medicine
5 Zaifu-cho, Hirosaki, 036-8562 JAPAN

Accepted
June 18, 2009

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