Classification, Diagnosis, and Management of.

Hilar Cholangiocar-cinoma, sometimes referred to as a Klatskin tumor, is a rare neoplasm arising proximal to the confluence of the cystic and common hepatic ducts. 1,2 In the United States, hilar cholangiocarcinoma has a reported annual incidence of 1 to 2 persons per 100 000 population. 3,4 Although a subset of patients may have a hilar tumor characterized as slow growing and late to.

Although hilar cholangiocarcinoma is relatively rare, it can be diagnosed on imaging by identifying its typical pattern. In most cases, the tumor appears to be centered on the right or left hepatic duct with involvement of the ipsilateral portal vein, atrophy of hepatic lobe on that side, and invasion of adjacent liver parenchyma.

Guidelines for the diagnosis and treatment of.

Cholangiocarcinomas (CCAs) are a heterogenous group of tumors that arise from the cholangiocytes that line the biliary tree. CCAs are classified based on their anatomic location, as follows: (1) intrahepatic CCA (iCCA), (2) perihilar CCA (pCCA), or (3) distal CCA (dCCA) (Figure 1). 1 iCCA is the second-most common primary liver cancer; its incidence has increased by 22% between 1979 and 2004.Infiltrating Hilar Cholangiocarcinoma.— Infiltrating hilar cholangiocarcinoma is the most common type of hilar cholangiocarcinoma (over 70% of cases). At contrast-enhanced CT, infiltrating tumors are seen as a focally thickened ductal wall obliterating the lumen (,,,,, Fig 6). About 80% of these tumors are hyperattenuating relative to the liver.However, the distinction between peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma is largely based on the site of origin. Therefore, in some tumors that arise peripheral to the secondary bifurcation of one of the hepatic ducts, clear differentiation between the two types of cholangiocarcinoma is not always possible.


Feydy A, Vilgrain V, Denys A, et al. Helical CT assessment in hilar cholangiocarcinoma: correlation with surgical and pathologic findings. AJR Am J Roentgenol 1999; 172:73. Tillich M, Mischinger HJ, Preisegger KH, et al. Multiphasic helical CT in diagnosis and staging of hilar cholangiocarcinoma. AJR Am J Roentgenol 1998; 171:651.Hilar cholangiocarcinoma. Medically reviewed by Drugs.com. Last updated on May 21, 2019. Disease Reference; Overview. Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder.

Cholangiocarcinoma is a relatively uncommon malignant neoplasm with poor prognosis. The distinction between extrahepatic and intrahepatic subtypes is important as epidemiological features, biologic and pathologic characteristics, and clinical course are different for both entities. This review study focuses on the role imaging plays in the diagnosis, classification, staging, and post-treatment.

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Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder. Hilar cholangiocarcinomas are also known as Klatskin tumors. Diagnosis. Tests and procedures used to diagnose hilar cholangiocarcinoma include.

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Abstract: Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6 th decade of life. Of the 3,000 cases seen annually in the United States, less than one half of these tumors are resectable. A variety of risk factors have been associated with HC, most notably primary sclerosing cholangitis (PSC), biliary stone disease and parasitic liver disease.

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Developed by renowned radiologists in each specialty, STATdx provides comprehensive decision support you can rely on - Cholangiocarcinoma.

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Intrahepatic cholangiocarcinoma is an epithelial malignancy arising from the intrahepatic biliary tree Recently divided into small duct type and large duct type, which have morphologic and molecular (but not prognostic) differences (Am J Surg Pathol 2018;42:1334, Surg Pathol Clin 2018;11:403).

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Welzel TM, McGlynn KA, Hsing AW, et al. Impact of classification of hilar cholangiocarcinomas (Klatskin tumors) on the incidence of intra- and extrahepatic cholangiocarcinoma in the United States. J Natl Cancer Inst 2006; 98:873.

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Another multidisciplinary approach for the treatment of advanced hilar cholangiocarcinoma is conversion therapy for the treatment of initially unresectable tumors. Several nonsurgical therapies are recommended for the treatment of locally advanced unresectable hilar cholangiocarcinoma as first-line therapies that provide prolonged overall survival and a substantial response ( 1, 50 ).

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Multimodality imaging of intrahepatic cholangiocarcinoma Intrahepatic cholangiocarcinomas account for approximately 20% of cases of cholangiocarcinomas. Three growth patterns or morphologic subtypes exist, including mass-forming, periductal-infiltrating, and intraductal-growth subtypes.

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Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. Light colored stool or dark urine may also occur. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater.

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Hilar cholangiocarcinoma (HCCA) is a rare cancer with a low resectability rate, frequent recurrence after resection and an overall poor outcome. It is widely accepted that en bloc partial hepatectomy is a necessary part of the surgical therapy, but controversy surrounds other areas, including extent of lymphadenectomy and preoperative use of biliary drainage of the future liver remnant (FLR).

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