Abstract
A 66-year-old woman presented to a physician with chief complaints of ash gray stool and epigastralgia. She was diagnosed with bile duct carcinoma and admitted to our hospital. Laboratory data showed abnormally high values for total bilirubin, serum hepatic transaminase and the tumor markers CA19-9 and DUPAN-2. Abdominal contrast computed tomography (CT) demonstrated an enhancing tumor in the middle portion of the common bile duct. Under a diagnosis of middle bile duct carcinoma, the patient underwent subtotal stomach preserving pancreatoduodenectomy and was discharged 30 days after the surgery without major complications. Immunohistochemically, neuroendocrine markers such as chromogranin A and synaptophysin were positive in the tumor cells. The tumor was diagnosed as primary endocrine cell carcinoma of the bile duct. The patient has not had a recurrence for nine months after the surgery, without adjuvant chemotherapy.
We present this rare case of primary endocrine cell carcinoma of the bile duct with a review of 12 cases reported in the Japanese literature.
