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Review
. 2021 Jun;113(3):158-169.
doi: 10.32074/1591-951X-252.

Cholangiocarcinoma

Samantha Sarcognato  1 , Diana Sacchi  1 , Matteo Fassan  2 , Luca Fabris  3 , Massimiliano Cadamuro  3 , Giacomo Zanus  4   5 , Ivana Cataldo  1 , Paola Capelli  6 , Francesca Baciorri  1 , Matilde Cacciatore  1 , Maria Guido  1   2
Affiliations
Review

Cholangiocarcinoma

Samantha Sarcognato et al. Pathologica. 2021 Jun.

Abstract

Liver cancer represents the third leading cause of cancer-related death worldwide. Cholangiocarcinoma (CCA) is the second most common type of liver cancer after hepatocellular carcinoma, accounting for 10-15% of all primary liver malignancies. Both the incidence and mortality of CCA have been steadily increasing during the last decade. Moreover, most CCAs are diagnosed at an advanced stage, when therapeutic options are very limited.

CCA may arise from any tract of the biliary system and it is classified into intrahepatic, perihilar, and distal CCA, according to the anatomical site of origin. This topographical classification also reflects distinct genetic and histological features, risk factors, and clinical outcomes. This review focuses on histopathology of CCA, its differential diagnoses, and its diagnostic pitfalls.

Keywords: biliary; cholangiocarcinoma; malignant; neoplasia; subtypes.

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Conflict of interest statement

Conflict of interest

The Authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
Large duct intrahepatic cholangiocarcinoma is composed of large irregularly dilated glands, embedded in abundant fibrous stroma. Note neoplastic cell mucus secretion (hematoxylin-eosin; original magnification 10x).
Figure 2.
Figure 2.
An example of perineural invasion in a large duct intrahepatic cholangiocarcinoma (hematoxylin-eosin; original magnification 10x).
Figure 3.
Figure 3.
A diffuse S100P expression in a large duct intrahepatic cholangiocarcinoma (S100P immunostain; original magnification 20x).
Figure 4.
Figure 4.
Small duct intrahepatic cholangiocarcinoma is composed of cuboidal cells arranged in small sized tubular or acinar structures, with areas of solid growth pattern, and with no mucin production (hematoxylin-eosin; original magnification 10x).
Figure 5.
Figure 5.
Cholangiolocarcinoma is made of malignant ductular-like structures, arranged in tubular, cord-like, anastomosing “antler-like” pattern, in a dense hyalinized fibrotic stroma (hematoxylin-eosin; original magnification 10x).
Figure 6.
Figure 6.
An intrahepatic cholangiocarcinoma with ductal plate malformation pattern, with tumor structures that look like ductal plate malformation, within a dense fibrotic stroma (hematoxylin-eosin; original magnification 2.5x).
Figure 7.
Figure 7.
A sarcomatoid intrahepatic cholangiocarcinoma, with diffuse spindle cell morphology. Numerous mitotic figures are present (A). As conventional intrahepatic cholangiocarcinoma, neoplastic cells diffusely express cytokeratin 7 (B) (A: hematoxylin-eosin; original magnification 20x; B: cytokeratin 7 immunostain; original magnification 20x).
Figure 8.
Figure 8.
Cholangioblastic variant of intrahepatic cholangiocarcinoma (A). Tumor cells diffusely express inhibin A (B) and show a granular cytoplasmic positivity for chromogranin A (C) (A: hematoxylin-eosin; original magnification 10x; B: inhibin A immunostain; original magnification 5x; C: chromogranin A immunostain; original magnification 20x).
Figure 9.
Figure 9.
Differently from benign lesions, intrahepatic cholangiocarcinoma shows a strong and diffuse nuclear expression of p53 (A), and a complete absence of p16 (B) (A: p53 immunostain; original magnification 20x; B: p16 immunostain; original magnification 20x).
Figure 10.
Figure 10.
Perihilar (A) and distal (B) cholangiocarcinomas share similar morphological features, being characterized by well-formed irregular neoplastic glands in a desmoplastic stroma (A: hematoxylin-eosin; original magnification 5x; B: hematoxylin-eosin; original magnification 5x).
See this image and copyright information in PMC

References

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