A CASE OF CHOLANGIO CELLULAR CARCINOMA WITH VON RECKLINGHAUSEN'S DISEASE

Abstract

A 33-year-old man who was born suffering from von Recklinghausen's disease was admitted to the hospital because of general fatigue and jaundice. On examinations, dilatation of intrahepatic bile ducts in the both lobes and a tumor mainly situating at the portal umbilicus were confirmed. To relieve obstructive jaundice, endoscopic naso biliary duct (ENBD) and percutaneous trash hepatic colangio drainage (PTCD) were conducted. However, it was impossible to insert PTCD into the right intrahepatic bile duct due to multiple neurofibromas on the skin. After close exploration, cholangio cellular carcinoma in stage IV was diagnosed. Although we could not expect to perform a radical operation, a left lobectomy of the liver with associated excision of the portal vein was performed for the purpose of improvement of the patient's QOL, tube free and volume reduction.
It is thought that volume reduction for bile duct cancer must be considered in cases in which extracorporeal drainage is impossible like in this case.