[Poorly differentiated large cell endocrine carcinoma of the extrahepatic bile ducts]

We report a case of a poorly differentiated endocrine large cell carcinoma of the extrahepatic bile ducts in a 73-year-old man, revealed by abdominal pain, jaundice and weight loss. Computed tomography and endoscopic retrograde cholangiography found tumoral stenosis of the main bile duct. Brush cytology detected tumor cells. Pathological examination of the resected bile duct disclosed a high-grade large cell carcinoma with morphological endocrine features and positivity for chromogranin A. This tumor was associated with a minor component of adenocarcinomatous cells. Despite polychemotherapy, the patient had widely metastatic disease a few months later. We discuss here the histogenesis of this tumor as well as its nosological position among the endocrine and mixed tumors of bile ducts.