Carcinoid tumors of the extrahepatic biliary tract: report of four cases.

Carcinoid tumors located in the biliary tree are exceedingly rare, accounting for 0.2%-2% of all gastrointestinal carcinoids. This study describes four cases of carcinoid tumors of the extrahepatic biliary tract. Four carcinoid tumors arising in the common bile duct (Case 1, 59-year-old man), gallbladder (Case 2, 49-year-old man; Case 3, 65-year-old man), and ampulla of Vater (Case 4, 52-year-old woman) were studied. All of the cases were misdiagnosed before surgery as proximal bile duct cancer, stomach cancer with liver metastasis, gallbladder cancer, and adenocarcinoma of ampulla of Vater, respectively. The clinicopathological characteristics and clinical course were reviewed. Treatment depends on the location of the tumor and the extent of the disease. Aggressive surgical therapy with a curative intention therefore offers the only chance for cure and has to be considered whenever possible.