BACKGROUND: There has been an increasing incidence and mortality from peripheral cholangiocarcinoma (PC) in the United States over the past 24 years. PC has been classified into two principal types, a mass-forming type and a periductal-infiltrating type, with a significant difference in the clinical behavior between the two. A third type, demonstrating a noninvasive intraductal growth of PC, was described as papillary PC. Rarely, papillary hepatic tumors composed of oncocytic cells have been described. Intraductal oncocytic papillary carcinomas (IOPCs) of the liver present as large, mucin-filled, cystic lesions lined by noninvasive or focally microinvasive oncocytic tumors. METHODS: From June 1999 to August 2001, three patients with hepatic IOPCs were identified in the files of the Hepatobiliary Service, Department of Surgery, and the Department of Pathology at Memorial Sloan-Kettering Cancer Center. They form the basis of this study. We report the clinicopathologic presentation, as well as the outcome, with a review of the literature. RESULTS: All three cases presented with well defined intrahepatic cystic masses ranging in size from 7.2 to 21.1 cm. The most prominent cells of the lining epithelium were columnar with oncocytic features showing abundant eosinophilic granular cytoplasm and centrally located nucleoli. All three patients underwent resection with one demonstrating local bile duct recurrence that was managed with stenting. Review of the literature has identified 39 patients with papillary PC and 2 patients with IOPC. The biology of these reported cases has been variable with overall survival better than that of nonpapillary PC patients, with recurrence in 15% of the reported cases. CONCLUSION: Papillary PC is a rare type of cholangiocarcinoma that includes an interesting variant: IOPC. These tumors are predominantly found in men, who present with large (> 5 cm) mucinous cystic lesions of the bile duct. A noninvasive histology is seen, and long-term survival may be achieved with complete resection. Invasive variants of IOPC have been reported in the literature and have a worse overall prognosis.
