Carcinoma of the pancreas associated with anomalous junction of pancreaticobiliary tracts: Report of two cases and review of the literature

Abstract

We report two cases of carcinoma of the pancreas with anomalous junction of the pancreaticobiliary tracts. A 71-year-old Japanese woman had obstructive jaundice. Ultrasonography showed a hypoechoic mass in the pancreatic head and computed tomography demonstrated a low-density nodule in the pancreatic head. Endoscopic retrograde cholangiopancreatography displayed a double duct sign and an anomalous junction of the pancreaticobiliary tracts. The patient underwent a pancreatoduodenectomy. The histopathologic diagnosis of the resected specimen was adenocarcinoma of the pancreatic head. A 56-year-old Japanese man also developed obstructive jaundice. Ultrasonography and computed tomography showed a huge mass almost replacing the whole pancreas and involving the superior mesenteric artery, splenic artery, splenic vein, and portal vein. Multiple hepatic metastases and peritoneal dissemination were present. Endoscopic retrograde cholangiopancreatography demonstrated an anomalous junction of the pancreaticobiliary tracts. The patient died of hemorrhage from esophageal varices. We discuss the relationship between the anomalous junction of the pancreaticobiliary tracts and pancreatic carcinoma.

Long-term Results of Treatment for Pancreaticobiliary Maljunction Without Bile Duct Dilatation

“…25 In the present study, 4 (9.1%) of 44 patients with PBM without bile duct dilatation had carcinoma of the pancreas, including the 2 patients previously reported. 25 The rate of association of pancreatic carcinoma was higher in the absence of bile duct dilatation in patients with PBM. The exact reason for this difference is not clear and remains to be investigated in the future.…”

Bile-Reflux into the Pancreatic Ducts is Associated with the Development of Intraductal Papillary Carcinoma in Hamsters

“…This result suggested the possibility of IPCs developing through activated pancreatic ductal cells with a high proliferating activity following an exposure to bile. In the PBM patients, the bile regurgitates into the pancreatic ducts under circumstances such as contraction of the gallbladder after meals, bile stasis in the choledochal cyst, the occurrence of cholangitis, or increased strain of the sphincter muscle of the papilla of Vater [6,20]. In such situations, the refluxing bile would gain a chance to produce carcinogenic effects on the pancreatic ducts.…”

“…Recently, some clinical reports have demonstrated the occurrence of intraductal papillary mucinous carcinoma (IPMC) in the main or large pancreatic ducts of patients with PBM. To our knowledge, at least 16 cases of pancreas carcinoma have been reported in patients with PBM [6,[12][13][14][15][16][17][18][19][20][21][22], and 5 (31%) of these tumors were noted to be IPMC histologically. Because IPMC accounts for only about 3% of all pancreas carcinomas [23,24], this clinical evidence suggests a strong relation between IPMC and PBM, supposing a hypothesis that bile-reflux into the pancreatic ducts may be a risk factor for the development of IPMC.…”

Self-expandable metallic stents for malignant biliary obstruction with an anomalous pancreaticobiliary junction

“…Patients with APBJ are not considered to be at high risk for pancreatic cancer. Nevertheless, a few reports have described patients with APBJ in whom pancreatic cancer developed [26,27], and the reported incidence of pancreatic cancer in APBJ patients is 1.9% to 4.5% [6,27].…”