Synchronous double primary squamous cell carcinoma and adenocarcinoma of the extrahepatic bile duct: a case report.

INTRODUCTION: Synchronous double cancers of the bile duct are exceptionally rare. We here report a case of synchronous squamous cell carcinoma and adenocarcinoma of the extrahepatic bile duct.

CASE PRESENTATION: A 67-year-old Asian man visited our clinic complaining of jaundice and dark urine. Direct hyperbilirubinemia and an elevated cancer antigen 19-9 level were detected. Preoperative abdominal computed tomography and positron emission tomography showed two masses at the bifurcation of the common hepatic duct and at the distal common bile duct. After biliary drainage, we performed radical pylorus-preserving pancreaticoduodenectomy, without resection margin involvement. Pathological findings revealed that the proximal lesion was a squamous cell carcinoma and that the distal lesion was an adenocarcinoma. Both cholangiocarcinomas were confined to the fibromuscular layer, and there was no communication between the two tumors. Multiple conglomerated metastatic tumors were detected in his liver 3 months after surgery. He died 8 months after diagnosis.

CONCLUSIONS: The disease displayed very aggressive behavior and a very poor prognosis. The only chance for long-term survival is treatment with radical resection. Preoperative positron emission tomography-computed tomography is useful in detecting occult cancer.