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Cholangiocarcinomas are tumors that arise from the bile duct epithelium anywhere from the liver to the ampulla of Vater. Intrahepatic cholangiocarcinoma accounts for 5–30% of all primary malignant hepatic tumors, and is the second most common primary malignant tumor of the liver after hepatocellular carcinoma (HCC). More than 90% of cholangiocarcinomas are adenocarcinomas. Other tumor types have been described. The histological grade of tumors varies from well-differentiated to undifferentiated. Most tumors consist of clusters of cells, surrounded by desmoplastic stroma, which can be extensive. The latter feature makes it difficult to distinguish between reactive tissue and well-differentiated cholangiocarcinoma. Furthermore, intrahepatic cholangiocarcinoma may be confused with metastatic scirrhous carcinoma on liver biopsy. Therefore, a primary adenocarcinoma as a source for metastases should be excluded when considering an intrahepatic cholangiocarcinoma.
Cholangiocarcinoma is more common in men than women, occurring most frequently between the sixth and seventh decades. Most patients have no predisposing risk factors, but primary sclerosing cholangitis (PSC) (5–15% lifetime risk), choledochal cysts (5% will transform and risk increases with age), Caroli's disease (7% lifetime risk), hepatolithiasis, chronic intraductal stones, bile duct adenoma, biliary papillomatosis, Clonorchis sinensis infection, and Thorotrast (thorium dioxide) exposure are some of the risk factors for cholangiocarcinoma. A higher prevalence of positive anti-hepatitis C virus has also been associated with cholangiocarcinoma.
Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) perform the major roles in the diagnosis of hepatocellular carcinoma (HCC) alongside alpha-fetoprotein (AFP) serology and biopsy. There is also increasing use of contrast-enhanced ultrasound (CEUS) for the diagnosis of HCC. Accessibility to each modality and expertise determine which is used as the major imaging tool for the diagnosis of HCC. In the context of the rapid increase in the incidence of HCC, it is important to recognize that there will be increasing reliance on imaging tools to diagnose and stage tumors.
Two sets of clinical practice guidelines published in the western literature form the basis of radiologic diagnosis of HCC in both Europe and the United States. These are the European Association for the Study of the Liver (EASL) guidelines formed at the single-topic conference on HCC in 2000 and the American Association for the Study of Liver Diseases (AASLD) practice guideline published in 2005.
The role of ultrasound is primarily in the surveillance of at-risk patients. The introduction of contrast agents has presented new opportunities for ultrasound specialists in the diagnosis of HCC. HCC shows strong intratumoral enhancement in the arterial phase followed by rapid washout with an isoechoic or hypoechoic appearance in the portal and delayed phases (Figure 4.1). Regenerative and dysplastic nodules do not show early contrast enhancement. Selective arterial enhancement has been shown in 91–96% of lesions confirming a high sensitivity in identifying the arterial neoangiogenesis of HCC.
The incidence of liver cancer in the United States and worldwide is increasing. The majority of primary liver cancers in the United States are hepatocellular carcinomas (HCC), with cholangiocarcinomas being the next most common. This trend is due to an increase in chronic hepatitis C, which along with hepatitis B is a major risk factor for liver cancer. Other contributing factors include heavy alcohol consumption, fatty liver disease, obesity, diabetes mellitus, and iron storage diseases. Although in general the mortality rates are high, survival rates in some countries are showing some improvement as more patients are being diagnosed with earlier stage tumors by means of aggressive surveillance with serologic tumor markers and diagnostic imaging. Advances in imaging techniques such as diffusion-weighted magnetic resonance imaging (MRI) and positron emission tomography–computed tomography (PET–CT) have helped in improving the detection and characterization of smaller earlier stage tumors. Treatment by means of resection or transplantation has excellent survival rates and, for patients who are not surgical candidates, ablative therapies and transarterial chemoembolization are suitable alternatives. Recently, for advanced HCC, anti-angiogenic agents have been employed with encouraging results. The role of radiotherapy in patients with cholangiocarcinoma and HCC who are poor surgical candidates is increasing.
The purpose of this edition of Contemporary Issues in Cancer Imaging is to review the epidemiology, screening, and diagnostic imaging techniques as well as roles of various therapeutic management strategies of common primary hepatic malignancies.
Primary carcinomas of the liver are increasing in incidence in the developed world, probably due to dietary and environmental factors as well as the known role of infective agents such as Hepatitis C. They are usually diagnosed in the advanced stages and carry high morbidity and mortality. This volume summarises the latest developments in imaging of primary hepatic carcinomas, emphasising the multidisciplinary approach to the care of patients. In addition to extensive chapters on the radiological issues surrounding diagnosis and staging of the malignancies, individual chapters on epidemiology, pathology, and surgical and non-surgical treatment options are included. All treatment options are considered for both HCC and cholangiocarcinoma, including newer techniques such as TACE. Only by a well orchestrated multidisciplinary team approach including epidemiologists, diagnostic radiologists, hepatologists, oncologic surgeons, radiation oncologists, and pathologists can these complex malignancies be managed successfully.
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