Intrahepatic cholangiocarcinoma

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

First presentation of decompensated liver failure.

Patient Data

Age: 70 years
Gender: Male

Ultrasound as initial investigation for liver failure

Modality: Ultrasound

The liver is enlarged and demonstrates a nodular surface. The liver echotexture is markedly coarse and nodular. No focal liver lesions found. The hepatic vein surface is slightly wavy. The hepatic and portal veins are patent and demonstrate normal direction of flow. The hepatic arteries are prominent, which most likely contributes to arterialised traces seen in the hepatic and portal veins (likely transmitted). No flow seen in ligamentum teres. The spleen measures 15.4 cm in craniocaudal length. The splenic vein is patent demonstrates normal direction of flow. Ascites is demonstrated.

Conclusion: Hepatosplenomegaly, cirrhosis and ascites. The hepatic and portal veins are patent.

Because the left lobe of the liver was so abnormal with a somewhat nodular exchotexture, the intial impression was that of cirrhosis, however there was no evidence of cirrhosis in the right lobe of the liver. Three litres of ascites were drained and were negative for abnormal cytology.

Modality: CT

There is an extensive abnormality involving the left lobe of the liver. This is composed of ill-defined, predominantly peripherally located hypoattenuating coalescing masses. No convincing enhancement is demonstrated. Vessels pass through this abnormality. There is associated capsular retraction in segment IVb anteriorly. In addition there is patchy differential enhancement in the liver in the portal venous phase between the left and right lobes. The right lobe is hypertrophied measuring up to 22.6 centimetres in maximum caudocranial dimension. No discrete arterially enhancing lesion is demonstrated. There is extensive ascites. Free fluid extends down both paracolic gutters into the pelvis. The SMV, splenic vein, and portal vein are patent. The spleen is large measuring up to 16.7 cm in maximal craniocaudal dimension. There is a simple cyst interpolar region of the left kidney. The right kidney, pancreas, and adrenal glands are unremarkable. Bowel is unremarkable. No concerning bone lesion is demonstrated. The lung bases are clear. Incidental note is made of a Morgagni hernia containing fat.

Impression: Features most in keeping with epithelioid haemangioendothelioma in the left lobe of liver. Peripheral cholangiocarcinoma is the main differential. MRI should be performed for further characterisation.

US with SMI (superb microvascular imaging)

Modality: Ultrasound

SMI demonstrates the internal vasculature in the left lobe of liver tumour.

Contrast enhanced ultrasound - guided core biopsy

Modality: Ultrasound

Definity contrast injection outlined a hypoechoic/hypoenhancing mass in segment II/III and IV which was barely distinguishable from liver on B-mode. Using a 17G/18G coaxial needle, samples were obtained from the hypoenhancing component (corresponding to abnormality seen on CT). Rapid on-site evaluation confirmed adequacy of specimens. No immediate complications.

Modality: Pathology

MACROSCOPIC DESCRIPTION: "Unlabelled as to site": Four cores of cream and dark red tissue 10- 14mm. A1. (SK) Biopsy attended and ROSE performed on specimen. Three air dried smears prepared and stained with rapid Romanovsky. Specimen adequate. MICROSCOPIC DESCRIPTION: Sections show cores of focally necrotic tumour composed of small irregular tubular structures in a dense fibrous stroma. The tubules are lined by cuboidal epithelial cells with pleomorphic hyperchromatic nuclei and small nucleoli. Some lumina contain mucin. In immunostains, the cells are CK7+, CK20-, CEA-, CDX-2-.

DIAGNOSIS: Core biopsy of lesion left lobe of liver: Adenocarcinoma; immunophenotype consistent with a cholangiocarcinoma.

Case Discussion

The tumour was so large that on initial US, diffuse liver disease, i.e. cirrhosis, was suspected. Contrast-enhanced ultrasound enabled targeted biopsy by delineating tumour well from normal liver, which was difficult on B-mode.  Without CEUS guidance, it is likely that multiple needle passes would have been required to achieve diagnostic material. Capsular retraction and hypoenhancement are features of cholangiocarcinoma.

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Case Information

rID: 31274
Case created: 28th Sep 2014
Last edited: 10th May 2016
Inclusion in quiz mode: Included

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