Abstract
A 79-year-old man presented to a physician with obstructive jaundice was referred to the hospital for further work-up. Abdominal contrast-enhanced computed tomography (CT) demonstrated an enhancing tumor in the distal portion of the common bile duct. Upper gastrointestinal endoscopy demonstrated a tumor mass on the papilla and carcinoma was detected from tissue obtained at a biopsy. Bile duct carcinoma was diagnosed. He underwent pancreatoduodenectomy and was discharged 32 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 distal bile duct. Abdominal CT scan performed 6 months after the surgery revealed multiple metastases in the liver. Although octreotide was given to the patient, the metastatic tumors in the liver rapidly progressed and he died 7 months after the surgery.
There have been only 26 reported cases, including our case, of primary endocrine cell carcinoma of the bile duct in Japan. The disease carries poor prognosis and no effective adjuvant chemotherapies have been established. Further accumulation of clinical cases would be mandatory.
