Biliary Papillomatosis Coexisting with Hepatitis C: Report of a Rare Case with Review of the Literature

Abstract

Biliary papillomatosis is a rare disease and is often fatal. It is characterized by diffuse papillary proliferations of bile duct epithelial cells. Patient may present with fever, abdominal pain and obstructive jaundice.  The degree of biliary obstruction varies, ranging from almost asymptomatic to severe life-threatening illness with painful obstructive jaundice, vomiting, cholangitis, and pancreatitis.  Preoperative diagnosis is very uncommon. Here, we describe an incidental finding of a case of biliary papillomatosis after liver transplant in a 65 year-old woman with end-stage liver disease secondary to Hepatitis C.  Grossly, the mass is located at the posterior aspect of the right lobe, extending to the portal area, presenting as a white fibrotic area measuring 6.0 x 8.0 x 4.5 cm.  Cut surface of this area is gray-white with multiple cysts ranging from 1.2 x 0.9 x 0.9 cm to 0.2 x 0.2 x 0.2 cm. Microscopic examination revealed glandular growth pattern of the epithelial cells with a stroma consisted of fibrovascular connective tissue.  These epithelial cells have abundant cytoplasm with the nuclei arranged regularly close to the basement membrane.  There is slight nuclear hyperchromatism, but there is no obvious atypia.  The tumor cells contain mucin as shown by PAS stain, which is resistant to the diastase treatment. The negative immunohistochemical stains for CEA and Cytokeratin 20 did not support the possibility of metastatic tumor from lower GI tract, while the positive Cytokeratin 7 staining was compatible with an origin of intrahepatic bile duct. Based on above findings, she was diagnosed multifocal biliary papillomatosis in the background of florid bile ductule proliferation. The cirrhosis and chronic inflammation in her liver were consistent with the history of hepatitis C. She also had associated chronic cholecystitis and cholelithiasis.  She was treated by liver transplantation.  The patient is doing well now (eight years after the transplantation).  In addition, the related literatures are reviewed, and the possible pathogenesis, treatment plan, and prognosis of biliary papillomatosis are briefly discussed.