Hepatobiliary and pancreatic: Papillary cholangiocarcinoma

Abstract

Papillary neoplasms of the bile duct are rare. Benign disorders include solitary biliary papillomas and multiple papillomas, either at the same time or at different times (biliary papillotomatosis). Both disorders are thought to be premalignant with the potential for evolution from adenoma to carcinoma. Carcinomas with papillary proliferation in the duct lumen are usually called papillary cholangiocarcinomas. These carcinomas have a distinctive appearance within the bile duct and, in contrast to typical cholangiocarcinomas, rarely show mutations in K-ras or p53 or aberrant expression of matrix metalloproteinase-2 or b-catenin. In addition, biliary papillary tumors appear to share pathological features with intraductal papillary mucinous neoplasms of the pancreas. The most common presenting symptoms are jaundice and pruritis. At endoscopic retrograde cholangiography or magnetic resonance cholangiography, the typical appearance is a dilated bile duct with extensive soft tissue expansion, often involving the common hepatic duct and intrahepatic ducts. A minority of patients have a mucus-filled papilla. Most patients are treated surgically with resection of the affected area, often including a partial hepatectomy. The prognosis is better than that of typical cholangiocarcinomas with a 5 year survival rate ranging from 35% to 60%. The patient illustrated below was a 64-year-old man whose liver function tests had become increasingly abnormal over the previous 2 years. He described fatigue and pruritis over the preceding 2 months. Five years previously, he had an uneventful laparoscopic cholecystectomy for gallstones. Tumor markers including carcinoembryonic antigen and CA19.9 were within the reference range. A computed tomography cholangiogram showed soft tissue expansion of the left hepatic duct extending down to the common hepatic duct. There was marked dilatation of the left hepatic duct and atrophy of the left lobe of the liver (Figure 1). At endoscopic retrograde cholangiography, gelatinous material was noted in a widely-open papilla. Biopsies were taken from the common hepatic duct after which a biliary stent was inserted. Biopsies did not reveal definite evidence of cancer. At laparotomy, choledochoscopy demonstrated a dilated bile duct filled with mucin while the left hepatic duct contained mucin and prominent papillary mucosal projections. The patient was treated with a left hepatectomy, radical resection of the extrahepatic biliary tree and reconstruction by hepatojejunostomy. Histology revealed mucus and papillary fronds covered by predominantly dysplastic intestinal-type epithelium (Figure 2) as well as a small focus of adenocarcinoma.