Cholangiocarcinoma - hilar type

Case contributed by Dr Bruno Di Muzio

Presentation

Obstructive jaundice.

Patient Data

Age: 70 years
Gender: Male
CT

CT Abdomen and pelvis

There is extensive intrahepatic duct dilatation, more pronounced in the left hepatic lobe. The common hepatic duct shows diffusely thick and enhancing walls. The gallbladder is contracted and shows an irregular enhancing mucosa. The common bile duct is difficult to visualize and hence not dilated. A few prominent but subcentimeter celiac lymph nodes are noted.

Normal enhancement of the pancreatic parenchyma on this study phase with no definite pancreatic head mass identified and the pancreatic duct is not dilated. No intra-hepatic lesion identified. The portal veins, splenic and superior mesenteric veins enhance normally.  No adenopathy elsewhere in the abdomen or pelvis. There are a small sliding-type hiatal hernia and an incidental splenunculus anterior to the spleen. The spleen, kidneys and adrenals are otherwise unremarkable.

The small and large bowel appear to be within normal limits. Widespread, severe cylindrical bronchiectasis at the imaged lung bases with a large number of bronchi either containing air-fluid levels or occluded by mucus. In addition, however, there are several small peripheral nodular densities which do not appear to be related to the bronchi and so are suspicious for pulmonary metastases.

 

MRI

MRCP

A hilar mass measuring 15 x 25 mm (transverse) is identified, resulting in marked intrahepatic duct dilatation. The stricture extends over a length of approximately 15 mm.
Gradual enhancement of the lesion is demonstrated on the dynamic series with diffusion restriction present. Beaking of the proximal left intra-hepatic duct with separation of the right anterior and posterior sectoral ducts.

No liver metastases. The common bile duct is normal.  No periportal or retroperitoneal lymphadenopathy. Portal vein and right and left branches are patent.  Hepatic veins are patent.
Gallbladder decompressed.

Annotated image

Features of a hilar cholangiocarcinoma involving the confluence of the hepatic ducts and common hepatic duct and extending to the bifurcation of the right hepatic duct.

Case Discussion

The imaging features are highly suggestive of a hilar cholangiocarcinoma (a.k.a. Klatskin tumor) involving the confluence of the hepatic ducts and common hepatic duct and extending to the bifurcation of the right hepatic duct, in keeping with a Bismuth-Corlette classification type 3A. 

Endoscopic brushings were sent to cytology: 

Microscopy: The smears consist predominantly of sheets of benign columnar epithelium with scant irregular clustered foci cells showing an enlarged nucleus, nuclear irregularities, irregular hyperchromasia and high nuclear to cytoplasmic ratio with ill-defined gland formation. The findings are in keeping with adenocarcinoma.

Pathologist opinion: Bile duct brushings–malignant.

The lung changes with bronchiectasis and inflammatory nodules were further worked up with CT chest and PET (not shown), involving predominantly the lower lobes, with no convincing metastatic disease identified.

 

 

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Case information

rID: 59780
Published: 5th Aug 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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