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Review
. 2017 Jan 15;11(1):13-26.
doi: 10.5009/gnl15568.

Cholangiocarcinoma: Current Knowledge and New Developments

Boris Blechacz  1
Affiliations
Review

Cholangiocarcinoma: Current Knowledge and New Developments

Boris Blechacz. Gut Liver. .

Abstract

Cholangiocarcinoma (CCA) is the second most common primary malignancy. Although it is more common in Asia, its incidence in Europe and North America has significantly increased in recent decades. The prognosis of CCA is dismal. Surgery is the only potentially curative treatment, but the majority of patients present with advanced stage disease, and recurrence after resection is common. Over the last two decades, our understanding of the molecular biology of this malignancy has increased tremendously, diagnostic techniques have evolved, and novel therapeutic approaches have been established. This review discusses the changing epidemiologic trends and provides an overview of newly identified etiologic risk factors for CCA. Furthermore, the molecular pathogenesis is discussed as well as the influence of etiology and biliary location on the mutational landscape of CCA. This review provides an overview of the diagnostic evaluation of CCA and its staging systems. Finally, new therapeutic options are critically reviewed, and future therapeutic strategies discussed.

Keywords: Bile duct; Cancer; Cholangiocarcinoma; Hepatobiliary; Neoplasia.

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Figures

Fig. 1
Fig. 1
Cholangiocarcinoma classification. Classification of cholangiocarcinoma based on its anatomic location within the biliary tree into intrahepatic, perihilar, and distal cholangiocarcinoma. Intrahepatic cholangiocarcinomas (iCCA) are located proximal to the secondary branches of the left and right hepatic ducts. Perihilar cholangiocarcinoma (pCCA) describes tumors located between the secondary branches of the right and left hepatic ducts and the common hepatic duct proximal to the cystic duct origin. Distal cholangiocarcinoma (dCCA) describes tumors of the common bile duct (CBD), up to but not including the ampulla Vateri. The dCCA within the intrapancreatic portion of the CBD can be difficult to distinguish from pancreatic head carcinomas. RA, right anterior segmental duct; RP, right posterior segmental duct; RHD, right hepatic duct; LHD, left hepatic duct; CHD, common hepatic duct; CD, cystic duct; GB, gallbladder.
Fig. 2
Fig. 2
Cholangiocarcinoma (CCA) histopathology. Characteristic histopathology of CCA (HE, ×20). Between 90% and 95% of CCA are adenocarcinomas of poor to moderate differentiation. Tumor cells are cuboidal to columnar and form glandular and tubular structures. Highly desmoplastic stroma and mucin are characteristic of CCA. (Courtesy of Dr. Wai Chin Foo, MD Anderson Cancer Center, Houston, TX, USA).
Fig. 3
Fig. 3
Bismuth-Corlette classification of perihilar cholangiocarcinoma (pCCA). Bismuth-Corlette classification of pCCA as types I to IV. The tumor is depicted in red, normal bile ducts are in green, and the cystic duct is in white.
Fig. 4
Fig. 4
Algorithm for the management and diagnosis of cholangiocarcinoma. (A) Algorithm for intrahepatic cholangiocarcinoma. (B) Algorithm for perihilar cholangiocarcinoma. CT, computed tomography; MRI, magnetic resonance imaging; HCC, hepatocellular carcinoma; iCCA, intrahepatic cholangiocarcinoma; CA 19-9, carbohydrate antigen 19-9; ERCP, endoscopic retrograde cholangiopancreaticography; FISH, fluorescent in situ hybridization; MRCP, magnetic resonance cholangiopancreatography; PET/CT, positron emission tomography/computer tomography; BC, Bismuth-Corlette; PSC, primary sclerosing cholangitis; OLT, orthotopic liver transplantation.
Fig. 4
Fig. 4
Algorithm for the management and diagnosis of cholangiocarcinoma. (A) Algorithm for intrahepatic cholangiocarcinoma. (B) Algorithm for perihilar cholangiocarcinoma. CT, computed tomography; MRI, magnetic resonance imaging; HCC, hepatocellular carcinoma; iCCA, intrahepatic cholangiocarcinoma; CA 19-9, carbohydrate antigen 19-9; ERCP, endoscopic retrograde cholangiopancreaticography; FISH, fluorescent in situ hybridization; MRCP, magnetic resonance cholangiopancreatography; PET/CT, positron emission tomography/computer tomography; BC, Bismuth-Corlette; PSC, primary sclerosing cholangitis; OLT, orthotopic liver transplantation.
Fig. 5
Fig. 5
Hepatocellular carcinoma (HCC) versus intrahepatic CCA (iCCA) on dynamic cross-sectional imaging. On quadruple phase imaging, iCCA (upper panel, white arrow) presents with progressive, heterogenous contrast-enhancement, whereas HCC (lower panel, white arrow) is characterized by homogenous contrast-enhancement followed by washout in the portal venous and delayed phases. Other radiologic iCCA characteristics include homogeneous low-attenuation mass with irregular peripheral enhancement, a lobulated morphology, hepatic capsular retraction, local vascular invasion, and proximal biliary dilatation. (Courtesy of Dr. Janio Szklaruk, MD Anderson Cancer Center, Houston, TX, USA).
Fig. 6
Fig. 6
Positron emission tomography/computer tomography (PET/CT) in the evaluation of metastatic disease in cholangiocarcinoma. A peritoneal metastasis in a patient with perihilar cholangiocarcinoma. The mass (arrow) was identified as a FDG-avid mass within the abdominal wall on PET/CT imaging (Courtesy of Dr. Janio Szklaruk, MD Anderson Cancer Center, Houston, TX, USA).
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