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Abstract and Introduction
Abstract
Cholangiocarcinomas are malignant tumors that derive from cholangiocytes of small intrahepatic bile ducts or bile ductules (intrahepatic cholangiocarcinoma; ICC), or of large hilar or extrahepatic bile ducts (extrahepatic cholangiocarcinoma; ECC). ICC and ECC differ in morphology, pathogenesis, risk factors, treatment, and prognosis. This review focuses on ICC, which is rising in incidence with the emergence of hepatitis C virus (HCV) infection as a risk factor. The authors examined 73 ICC, which were resected at The Mount Sinai Medical Center in New York City, and reviewed the literature. The tumors were categorized into classical and nonclassical ICCs based on histopathology. Classical ICCs (54.8%) were characterized by a tubular, glandular, or nested pattern of growth, were significantly associated with tumor size of more than 5 cm and the absence of underlying liver disease and/or advanced fibrosis. Nonclassical ICCs (45.2%) consisted of tumors with trabecular architecture, tumors that exhibited features of extrahepatic carcinomas, and carcinomas considered to be derived from hepatic progenitor cells, i.e., combined hepatocellular/cholangiocarcinomas and cholangiolocellular carcinomas (ductular type of ICC). They were smaller and often arose in chronic liver disease, mostly HCV infection, and/or with significant fibrosis. The role of immunohistochemistry in the diagnosis of ICC and the importance of the new American Joint Committee on Cancer Staging System for ICC are also discussed.
Introduction
Cholangiocarcinomas are adenocarcinomas that arise from the malignant transformation of bile duct epithelium anywhere along the biliary tree from small bile ducts and bile ductules (intrahepatic cholangiocarcinomas; ICCs), to large bile ducts at the hilum of the liver or outside the liver (extrahepatic cholangiocarcinomas; ECCs). Nomenclature of bile duct tumors is still a matter of debate. It has been proposed that the term "cholangiocarcinoma" be reserved for intrahepatic peripheral lesions and tumors arising from large bile ducts both at the hilum and along the extrahepatic biliary tree, be designated "bile duct carcinomas." Indeed, hilar carcinomas, often called Klatskin tumors are more similar to ECCs than to ICCs. Together with the other extrahepatic bile duct carcinomas they account for ~80% of all cholangiocarcinomas. They affect men more than women with a peak age in the sixth or seventh decade and are often detected at an early stage with signs of biliary obstruction. In contrast, ICCc typically occur in patients in the fifth or sixth decade of life, with no frank sex predilection and patients remain asymptomatic for a longer period. For this reason, the median survival for ICC is short, ~6 months without surgery; and the 5-year survival rate is only 20 to 40% following resection, even with clear margins. ICCs and ECCs also differ in morphology, molecular background, and treatment management. This review focuses on our experience at The Mount Sinai Medical Center in New York City with surgically resected ICC, and the most recent literature.
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