Clinical diagnosis and staging of cholangiocarcinoma

Abstract

Cholangiocarcinoma is the most frequent biliary malignancy. It is difficult to diagnose owing to its anatomic location, growth patterns and lack of definite diagnostic criteria. Currently, cholangiocarcinoma is classified into the following types according to its anatomic location along the biliary tree: intrahepatic, perihilar or distal extrahepatic cholangiocarcinoma. These cholangiocarcinoma types differ in their biological behavior and management. The appropriate stratification of patients with regard to the anatomic location and stage of cholangiocarcinoma is a key determinate in their management. Staging systems can guide this stratification and provide prognostic information. In addition, staging systems are essential in order to compare and contrast the outcomes of different therapeutic approaches. A number of staging systems exist for cholangiocarcinoma-several early ones have been updated, and new ones are being developed. We discuss the emerging diagnostic criteria as well as the different staging systems for cholangiocarcinoma, and provide a critical appraisal regarding these advances in biliary tract malignancies.

Figure 1

Cholangiocarcinoma subtypes. Cholangiocarcinoma is classified as either intrahepatic or extrahepatic, with the second-order bile ducts acting as the separation point. Classically, extrahepatic cholangiocarcinoma has been divided in perihilar and distal extrahepatic cholangiocarcinoma at the level of the cystic duct.

Figure 2

Intrahepatic cholangiocarcinoma growth types. The Liver Cancer Study Group of Japan (LCSGJ) used retrospective data from 245 patients to distinguish three macroscopic growth types for intrahepatic cholangiocarcinoma: mass-forming type; periductal-infiltrating type; and intraductal-growth type.

Figure 3

Histpathological features of mucin-producing cholangiocarcinoma and cholangiolocellular carcinoma with mixed features. a | Well differentiated intrahepatic cholangiocarcinoma. Tubular structure with abundant fibrous stroma. b | Cholangiolocellular carcinoma. Ductular-reaction-like structure with fine edematous fibrous stroma. c | Hepatocellular-carcinoma-like area in cholangiolocellular carcinoma. d | Mucin-producing perihilar cholangiocarcinoma showing perineural invasion.

Figure 4

Algorithm for the diagnosis and management of intrahepatic cholangiocarcinoma.

Figure 5

Diagnostic criteria for perihilar and distal extrahepatic cholangiocarcinoma.