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Review
. 2022 Dec 26;30(1):370-380.
doi: 10.3390/curroncol30010030.

Pathology of Cholangiocarcinomas

Nathalie Guedj  1
Affiliations
Review

Pathology of Cholangiocarcinomas

Nathalie Guedj. Curr Oncol. .

Abstract

Cholangiocarcinomas (CCA) are heterogeneous tumors that arise from epithelial cells of the biliary tract. They represent the second primary liver malignancy, after hepatocellular carcinoma. Recent epidemiological data show an increased incidence of intrahepatic CCA without any identified causes. According to their location on the biliary tract, intrahepatic, perihilar (p) and distal (d) CCA can be individualized. Intrahepatic CCA (iCCA) are subdivided into small duct type iCCA and large duct type iCCA, according to the level or size of the biliary duct affected. These two subgroups are characterized by distinct risk factors, gross aspect, histopathological and molecular features, and therapeutic management. The role of biopsy in iCCA is to confirm the diagnosis and to eliminate various differential diagnostics, in particular, metastases. In p/d CCA, biopsy requires more invasive approaches, and tissue samples are difficult to obtain, leading to a high rate of false negatives. In this review, we will discuss the different classifications of CCA (anatomical and macroscopic). We will describe the various microscopic and phenotypic subtypes of CCA. Finally, we will deal with their mode of extension, the role of biopsy and pre-neoplastic lesions.

Keywords: biliary duct; cholangiocarcinoma; liver.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Localization, distribution and frequency of the macroscopic type of cholangiocarcinoma. MF: maa forming, PI: periductal infiltrating, IG: intraductal growth.
Figure 2
Figure 2
Macroscopic classification of cholangiocarcinoma: (A) mass-forming type of an intrahepatic cholangiocarcinoma, (B) periductal infiltrating of perihilar cholangiocarcinoma and (C) intraductal growth of left biliary duct.
Figure 3
Figure 3
Microscopic aspects of intrahepatic cholangiocarcinoma: (A) presence of a desmoplastic stroma, (B) small duct type, (C) cholangiolocarcinoma, and (D) ductal plate cholangiocarcinoma (HES staining ×100).
Figure 4
Figure 4
Large duct intrahepatic cholangiocarcinoma (HES staining ×250).
Figure 5
Figure 5
Microscopic aspect of periductal infiltrating (A) and intraductal growth (B) cholangiocarcinoma (HES staining ×100).
Figure 6
Figure 6
Immunohistochemistry of CK7 (A) and CK19 (B) showing strong cytoplasmic staining (brown) of an intrahepatic cholangiocarcinoma.
Figure 7
Figure 7
Biliary preneoplastic lesions: (A) flat high-grade biliary intraepithelial neoplasia (BilIN); (B) low-grade intraductal papillary neoplasia (IPBN) (HES staining ×250).
See this image and copyright information in PMC

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