Anomalous pancreaticobiliary junction (pancreaticobiliary maljunction)
A birth variation in which the bile duct and pancreatic duct join outside the wall of the bowel, so pancreatic juice flows back up into the bile ducts and gallbladder and irritates their lining for life. It carries a high risk of gallbladder cancer, and removing the gallbladder is advised once it is found.
Overview
In pancreaticobiliary maljunction the two ducts unite outside the duodenal wall to form a long common channel not controlled by the sphincter of Oddi, allowing reflux of pancreatic enzymes into the biliary tree; it often coexists with a choledochal cyst, and Cancer Research UK lists both as congenital risk factors for gallbladder cancer, quoting about seven times the risk for the maljunction from a meta-analysis of case-control studies (Deng 2011). It is usually diagnosed on MRCP by measuring the common channel and the narrow distal segment. In a 2025 Japanese MRCP series the maljunction was found in 0.44 percent of 2,046 benign cholecystectomies but in 16 percent of gallbladder cancers and 1.3 percent of bile duct cancers; a milder variant, high confluence of the pancreaticobiliary ducts, was present in about 1 percent of the population and 4.2 percent of gallbladder cancers. Combining prevalence with national cancer data, the authors estimated a 2.4 percent ten-year incidence of gallbladder cancer with the maljunction, 38 times the general population (Shirai 2025). That figure is the argument for removing the gallbladder, and a dilated bile duct where present, once the anomaly is found; Cancer Research UK lists the maljunction and choledochal cysts among the congenital risk factors and notes most people with these conditions develop symptoms in childhood, some only as adults.
- Pancreatic head (most PDAC)
- Body and tail
- Ampulla
- Islets (pancreatic NET)
- Intrahepatic ducts
- Perihilar (Klatskin)
- Distal bile duct
- Gallbladder
- Nodes: peripancreatic
- Nodes: hepatic hilar
- Nodes: coeliac and superior mesenteric
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
- Pancreatic head (most PDAC)
- Body and tail
- Ampulla
- Islets (pancreatic NET)Neuroendocrine carcinoma of the gallbladder (small cell and large cell; under 5 percent)
- Intrahepatic ducts
- Perihilar (Klatskin)
- Distal bile duct
- GallbladderGallbladder adenocarcinoma (about 90 percent; biliary, intestinal, mucinous and other patterns) · Papillary carcinoma of the gallbladder, and its precursor the intracholecystic papillary neoplasm · Mucinous carcinoma of the gallbladder (about 2.5 percent) · Adenosquamous and squamous carcinoma of the gallbladder (about 5 percent) · Neuroendocrine carcinoma of the gallbladder (small cell and large cell; under 5 percent) · Incidental gallbladder cancer found in the cholecystectomy specimen · Carcinoma in situ and dysplasia of the gallbladder (biliary intraepithelial neoplasia; stage 0) · Cystic duct carcinoma (staged with gallbladder cancer)
- peripancreatic
- hepatic hilar
- coeliac and superior mesenteric
Same organ: Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma
Showing the organ this term concerns: Gallbladder cancer.
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