Carcinoma of the Papilla of Vater after Diversion Operation for Pancreaticobiliary Maljunction

Abstract

Pancreaticobiliary maljunction (PBM) is a congenital malformation that is associated with biliary cancer development. When patients are diagnosed with PBM, a diversion operation is recommended. Although a risk remains for developing residual bile duct carcinoma following diversion, the development of a carcinoma of the ampulla of Vater after a diversion operation for PBM is rare. We present a treated case of carcinoma of the ampulla of Vater after a diversion operation for PBM. A 65-year-old woman presented with abdominal pain. She had undergone extrahepatic bile duct resection and cholecystectomy 2 years 9 months previously for the treatment of type Ic PBM according to the Todani classification. At the current admission for evaluation of the abdominal pain, computed tomography and magnetic resonance imaging showed only dilation of the main pancreatic duct. However, gastrointestinal endoscopy showed a tumor at the papilla of Vater, and biopsy revealed adenocarcinoma of the papilla of Vater. We performed pylorus-preserving pancreaticoduodenectomy, and the pathological diagnosis was moderately differentiated tubular adenocarcinoma of the papilla of Vater with no metastasis to the lymph nodes. The patient remained in good health for 3 years postoperatively. Carcinoma of the papilla of Vater after a diversion operation for PBM is rare. In this case, a diagnosis could not be made by computed tomography or magnetic resonance imaging; the definitive diagnosis was obtained with gastrointestinal endoscopy. Careful postoperative follow-up with gastrointestinal endoscopy in addition to imaging examination may be needed after a diversion operation for PBM.

A case report of carcinoma of the papilla of Vater associated with a hyperplasia–dysplasia–carcinoma sequence by pancreaticobiliary maljunction

“…Therefore, the mucosal damage in the common duct is considered to be persistent. Previous studies have focused on PVca with PBM, though it remains unclear whether PBM is the cause of carcinogenesis in any of the previous studies [ 5 , 6 , 13 – 17 ]. PVca is classified as cholangiocarcinoma, and the usual carcinogenic process underlying PVca is the adenoma–carcinoma sequence [ 18 ].…”

“…Residual bile duct cancer has been reported to be detectable during long-term follow-up, even after termination of pancreaticobiliary reflux [ 3 , 4 ], and 23 (1.8%) of 1291 patients developed residual bile duct cancer after cyst excision [ 4 ]. In addition to residual bile duct cancer, carcinoma of the papilla of Vater (PVca) has been reported; however, it is considerably rare [ 5 , 6 ]. Although the risk factors for PVca have not been clarified [ 7 ], we report our experience with PVca that was thought to be caused by the hyperplasia–dysplasia–carcinoma sequence, which is considered a mechanism underlying PBM-related biliary tract cancer.…”

Application of imaging techniques in pancreaticobiliary maljunction

“…Pancreaticobiliary maljunction (PBM) was first recorded in the early 20 th century, and officially named in 1969, which is referred to as congenital malfuntional[ 1 ]. The main anatomical feature of PBM is that the bile duct and pancreatic duct join out of the duodenal wall, forming a lengthy common duct (Figure 1 )[ 2 , 3 ], often combined with sphincter of Oddi dysplasia. As a result, the pancreatic duct and bile duct lose control, causing reflux[ 4 ].…”