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Primary liver and biliary tumors are a significant health threat globally.
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These tumors have wide geographic, ethnic, and gender variation.
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Research investigating the epidemiology and risk factors associated with these tumors has resulted in significant global public health measures to reduce incidence rates.
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Recently identified risk factors, such as metabolic disorders, show the important role that epidemiology will continue to have in the understanding and treatment of these tumors.
Epidemiology and Risk Factors of Biliary Tract and Primary Liver Tumors
Section snippets
Key points
Hepatocellular cancer
Hepatocellular cancer (HCC) is the most common form of primary liver cancer, accounting for 85% to 90% of all primary liver cancers, with the burden of disease expected to increase in coming years, especially in the developing world.1 It is the fifth most common cancer worldwide and the third most common cause of cancer mortality, accounting for more than 600,000 deaths annually.2 HCC was one of the first cancers to be linked epidemiologically to a defined risk factor (hepatitis B virus [HBV]
Hemangioma
Hepatic hemangioma is the most common benign lesion of the liver, with an estimated prevalence of 5% to 20% in the general population.23 Hepatic hemangiomas are vascular abnormalities characterized by blood-filled, sinusoidal spaces lined with endothelium. It is thought that dilatation of these spaces, and not angiogenesis, causes enlargement over time. These lesions are incidentally found on abdominal exploration and on imaging studies. Most hepatic hemangiomas are identified in patients
Focal nodular hyperplasia
Focal nodular hyperplasia (FNH) is a benign, indolent tumor of the liver with no known malignant potential. It is the second most common benign liver tumor after hepatic hemangiomas. FNH is reported to occur with a frequency between 0.31% and 3%.26, 27 It is more commonly identified incidentally in young women between the ages of 30 and 50 years.27, 28 The female/male ratio has been reported to be between 8:1 and 12:1.27, 29 FNH is rarely found in the pediatric population and the elderly. The
Hepatocellular adenoma
Hepatocellular adenoma is a noncancerous neoplasm of the liver, but, unlike liver hemangiomas and FNH, it has malignant potential. Hepatic adenoma is usually a solitary lesion that on histologic analysis consists of normal to slightly enlarged hepatocytes arranged in a trabecular pattern. The pathogenesis is strongly associated with oral contraceptive use.32 In North America and Europe, the incidence is about 0.1 per 100,000 in non–oral contraceptive users and 3 to 4 per 100,000 in oral
Gallbladder cancer
Gallbladder cancer is the fifth most common cancer involving the gastrointestinal tract, but the most common malignant tumor of the biliary tract worldwide. When diagnosed, it is typically found incidentally during cholecystectomy. However, gallbladder cancer frequently presents with vague symptoms and, as a result, its diagnosis occurs at an advanced stage. The late stage of presentation results in patients showing both locally advanced as well as metastatic spread. The overall mean survival
Cholangiocarcinoma
Cholangiocarcinoma, or malignant strictures of the biliary tract, arises from the ductal epithelium of the biliary tree, either within the liver substance (intrahepatic) or outside the liver (extrahepatic). It is a disease that, when detected early, is amenable to surgical resection. However, in many instances, it is discovered at a locally advanced or widely disseminated stage and as a result portends a poor prognosis. The molecular mechanisms involved in malignant transformation of biliary
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