Malignant papillomatosis of the intrahepatic bile ducts

A case of intrahepatic bile duct papillomatosis is reported which recurred several times and finally progressed into a mucin-secreting papillary adenocarcinoma. The paillomatosis predominantly involved the ductal system of the left hypoplastic hepatic lobe. The treatment consisted of a left partial hepatectomy. Hereby, a four-year survival was reached. While the prognosis of solitary papillomas in the distal choledochus and in the gallbladder is considered as good, the prognosis of the intra- and extrahepatic bile duct papillomatosis appears unfavorable. The experience in this case, as well as in the literature, shows that these papillomatoses have to be regarded as low-grade carcinomas.