Oncocytic Biliary Cystadenocarcinoma Is a Form of Intraductal Oncocytic Papillary Neoplasm of the Liver

Abstract

Biliary cystadenocarcinoma with oncocytic differentiation was first reported in 1992. This is a report of a second case. The patient (a 71-year-old man) was admitted to our hospital complaining of abdominal fullness. Multicystic lesions were identified in the left hepatic lobe radiologically. The patient died of peritoneal dissemination of carcinoma 20 months later. At autopsy, the tumor of the left hepatic lobe was found to be composed of adjoining multiple cystic lesions and a solid lesion with infiltration of the hepatic hilus and peritoneal dissemination. Histologically, the multicystic lesions were covered by papillary neoplastic epithelial cells with an eosinophilic granular cytoplasm resembling that of oncocytes and a fine fibrovascular core. The cyst wall was fibrous, but there was no mesenchymal stroma. In the solid lesion and infiltrated areas, acidophilic and granular carcinoma cells formed small glandular or solid cord patterns with much mucin secretion (mucinous carcinoma). Immunohistochemically, carcinoma cells of both components were found to contain many mitochondria and showed the phenotypes of hepatocytes and cholangiocytes. Interestingly, the intrahepatic biliary tree also was invaded by carcinoma cells. This may be a case of intraductal oncocytic papillary neoplasm of the left hepatic lobe followed by secondary cystic dilatation of the affected bile duct.

Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct

“…From the view point of papillary proliferation, discrimination between IPN-B and biliary papillomatosis or cholangiocarcinoma of intraductal growth-type might be a problem. [29][30][31][32][33] We now speculate that most of biliary papillomatosis and cholangiocarcinoma of intraductal growth-type can be regarded as IPN-B, although a detailed clinicopathological study using a large numbers of cases is mandatory to conclude this issue. Biliary tumors with abundant mucin production have been called mucin-hypersecreting bile duct tumors.…”

Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas

“…28 Similarly, Adsay et al reported that two types of tumor progression could occur in IPMN-P: the MUC1-positive pathway to tubular adenocarcinoma and the MUC2-positive pathway to mucinous carcinoma. 29 There have been already several reports revealing close similarities between clinical, pathological and radiological characteristics of biliary papillary tumors and IPMN-P. 12,14,[30][31][32][33][34] This study, using a considerable number of biliary papillary tumors and IPMN-P, also showed sufficient evidence that biliary papilloma(tosis) and papillary-CC with or without mucus hypersecretion belong to a single tumor entity of intraductal papillary neoplasm of the bile duct, as a biliary counterpart of IPMN-P. Intraductal papillary neoplasm of the bile duct could manifest clinicopathologically as papilloma, papillomatosis and papillary adenocarcinoma (papillary-CC) (in situ and invasive). However, the distinction of papillary-CC in situ from biliary papilloma(tosis) is still controversial.…”

Primary and Metastatic Tumours of the Liver: Expanding Scope of Morphological and Immunohistochemical Details in the Biopsy

“…Hypothetically, these tumours arise from bile ducts proximal to the hilum of the liver and share the cystic structure and presence of peculiar ovarian-type mesenchymal stroma with mucinous cystic tumours of the pancreas and retroperitoneum, leading to the hypothesis that ectopic ovarian stroma during embryogenesis can become incorporated along the biliary tree, in the pancreas and retroperitoneal space and cause the proliferation of the adjacent epithelium by production of the hormones and growth factors [121]. Origin from intrahepatic peribiliary glands [122] or from ectopic rests of primitive foregut sequestered in the liver [114] has been hypothesised. Development from pluripotential stem cells is suggested on the basis of the presence of albumin messenger RNA and biliary type cytokeratins in the tumour cells [123].…”

“…Development from pluripotential stem cells is suggested on the basis of the presence of albumin messenger RNA and biliary type cytokeratins in the tumour cells [123]. Biliary cystadenocarcinoma without mesenchymal stroma more frequently arises in males and carries poorer prognosis in comparison with the tumour possessing mesenchymal stroma [122]. By immunohistochemistry, increasing proliferative activity by Ki-67 ex-pression as well as increasing p53 protein expression from adenoma to carcinoma was shown in biliary cystadenocarcinoma without ovarian-type stroma [124].…”