Hepatic epithelioid hemangioendothelioma with pulmonary metastasis

Case contributed by Husam Hussein Yaseen
Diagnosis certain

Presentation

Chronic upper abdominal pain with a one-month history of jaundice.

Patient Data

Age: 45 years
Gender: Female

Infiltrative hypoechoic mass involving a large area of the liver parenchyma.

A pre- and post-contrast CT scan reveals a large infiltrating hepatic hypodense lesion with multiple other satellite lesions that are peripherally enhanced.

Dilated and probable thrombosed intrahepatic portal branches related to the effect of an infiltrative lesion.

Bilobed, markedly enlarged (hypertrophied) caudate lobe.

Multiple small pulmonary nodules were seen in the lower lobes.

The hepatic lesion is hypointense in T1 and has intermediate hyperintensity in T2, with numerous hyperintense foci inside.

The portal vein is tapered at its branching site (lollipops sign).

Case Discussion

Histopathology

The histopathology slides show cords or single atypical cells with a polygonal to spindle shape and abundant oncocytic cytoplasm with occasional intracytoplasmic vacuoles ("blister cells") with an infiltrative growth pattern in myxohyaline/fibrous stroma. The presence of well-formed vascular channels and areas of solid growth of epitheloid cells with eosinophilic cytoplasm and occasional non-neoplastic hepatocytes within normal histologic limits. A picture is suggestive of a primary liver malignancy (suggestive of epitheloid hemangioendothelioma).

An immunohistochemical study (CD34, CK7, and CK19) is recommended to confirm the diagnosis and to exclude another differential diagnosis: the fibrolamellar variant of HCC. The immunohistochemical study reveals positive CD 34 and negative CK 7 and CK 19, which are consistent with epitheloid hemangioendothelioma.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.