Radiologic Spectrum of Cholangiocarcinoma: Emphasis on Unusual Manifestations and Differential Diagnoses

Abstract

Most cholangiocarcinomas are ductal adenocarcinomas that arise from both intra- and extrahepatic bile duct epithelium, and their typical growth pattern can be classified as exophytic, infiltrative, polypoid, or a combination of these. Those of unusual histologic type (eg, mucin-hypersecreting cholangiocarcinoma, squamous adenocarcinoma, biliary cystadenocarcinoma, and mucinous carcinoma) show a growth pattern different from that of the typical ones (ie, ductal). Cholangiocarcinomas frequently develop in patients with any of a variety of preexisting bile duct diseases, some of which are considered precursors of cholangiocarcinoma (eg, biliary lithiasis, clonorchiasis, recurrent pyogenic cholangitis, and primary sclerosing cholangitis). Some bulky hepatic tumors of either primary or secondary origin mimic exophytic peripheral cholangiocarcinoma. Some variants of hepatocellular carcinoma, such as sclerosing, fibrolamellar, and cholangiohepatocellular carcinoma, resemble exophytic peripheral cholangiocarcinoma, while that with intraductal growth resembles polypoid cholangiocarcinoma. Among benign bile duct diseases, tumorous conditions (eg, benign biliary tumors) may mimic polypoid cholangiocarcinoma, whereas benign stricture of various causes (eg, cholangitides, traumatic and postsurgical sequelae, chronic pancreatitis, papillary stenosis) usually mimics infiltrative cholangiocarcinoma.

CT Differentiation of Cholangiocarcinoma from Periductal Fibrosis in Patients with Hepatolithiasis

“…However, only a few reports describe the radiologic findings of cholangiocarcinoma occurring in patients with hepatolithiasis [17][18][19]. In patients with hepatolithiasis, fibrotic masses in the bile duct walls and periductal hepatic parenchyma are often seen as a form of periductal tumorlike lesions and may appear similar to cholangiocarcinoma on CT [19].…”

Focal Liver Lesions

“…At CT and MR imaging, lesions tend to be hypodense at unenhanced CT and hypointense on T1-weighted images, with peripheral enhancement at dynamic contrast-enhanced studies [67]. Delayed phase CT/MR imaging (after 5-15 min) may show enhancement homogeneously or in the center of the lesion due to its rich fibrous stroma, which is suggestive of the diagnosis of CCC [68]. Interestingly, the central fibrotic stroma often shows signal suppression on diffusion-weighted MRI and return relatively high ADC value (Fig.…”

Mass-forming intrahepatic cholangiocarcinoma presenting with painful obstructive hemobilia

“…BernardSoulier syndrome, idiopathic thrombocytopenic purpura, hemophilia and treatment with anticoagulants) Based on gross anatomic morphologic characteristics, cholangiocarcinoma are classified into massforming, periductal infiltrating and intraductal grow type (10,12). Traditionally, extrahepatic bile duct cancer has been classified as nodular, sclerosing, or papillary, corresponding to the mass-forming, periductal infiltrating, and intraductal growth types of intrahepatic cholangiocarcinoma, respectively (10,(12)(13). This classification is useful for the interpretation of the imaging findings and for the differential diagnosis but also for prediction of dissemination, prognosis and planning of surgery (10).…”

“…Recognized risk factors for cholangiocarcinoma all share the common feature of chronic biliary inflammation (10)(11)(12). Schistosomiasis and hepatolithiasis are common risk factors in eastern Asia, whereas primary sclerosing cholangitis, liver cirrhosis, alcohol-related liver disease, and diabetes are relatively common risk factors in Western countries (10).…”

“…Schistosomiasis and hepatolithiasis are common risk factors in eastern Asia, whereas primary sclerosing cholangitis, liver cirrhosis, alcohol-related liver disease, and diabetes are relatively common risk factors in Western countries (10). Various viral infections, anomalies and/or malformations of the biliary tract, biliary-enteric drainage procedures and environmental or occupational toxins also constitute risk factors (10,12). be directly estimated and opacification of the biliary tree can reveal the causes of hemobilia, the type and site of filling defects, and degree of bile duct dilatation (2).…”