Synchronous double primary cancers of the extrahepatic bile duct: A case report and literature review

Abstract Background Double cancers of the biliary tract system are rare. Most of these cancers are synchronous double cancers of the gall bladder and bile duct, associated with pancreaticobiliary maljunction (PBM). Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and only 4 cases have been reported. Case presentation A 78-year-old woman was admitted to our hospital for examination of hyperbilirubinemia and liver dysfunction. Contrast-enhanced abdominal computed tomography, Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed 2 stenotic regions in the common bile duct: at its junction with the cystic duct and in the distal bile duct. No findings suggested PBM, such as a markedly long common channel. The diagnosis based on endoscopic brush cytology from both stricture portions was adenocarcinoma. The patient had a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection. Macroscopically, there were 2 stenotic regions at the cystic duct junction and in the distal bile duct. Microscopically, the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma. On the other hand, the tumor of the distal bile duct was a poorly differentiated adenocarcinoma. There was no evidence of communication between these 2 cancers. Conclusion Double cancers of the extrahepatic bile duct without PBM are very rare. Therefore, an accurate diagnosis prior to surgery is necessary. Furthermore, this rare condition seems to be associated with a poor prognosis.