Hepatic and pulmonary epithelioid hemangioendothelioma

Case contributed by Dalia Ibrahim
Diagnosis certain

Presentation

Incidental hepatic focal lesions on abdominal US.

Patient Data

Age: 50 years
Gender: Female

Liver:

Multiple bilobar FDG-avid variable sized hepatic focal lesions. The most metabolically active focal lesion is seen at segment VII with SUV max 4.0. The lesions show marginal (halo/target pattern) contrast enhancement. Some lesions show internal calcifications.

Lungs:

Multiple small scattered pulmonary nodules showing mildly increased FDG uptake.

Spleen:

Non-FDG avid small non-enhancing focal lesion seen at its upper pole.

Multiple bilobar hepatic focal lesions eliciting low signal on T1WI and high signal on T2WI with "white target sign" formed by high-signal intensity core and peripheral slightly hyperintense halo. The focal lesions show diffusion restriction. The lesions are predominantly peripherally located and subcapsular, with one intra-parenchymal lesion in segment VIII. The right hepatic focal lesion seen at segment VI causes adjacent capsular retraction evident on the coronal images. The large focal lesions show internal rings of low signal.

A similar yet smaller lesion is seen in the spleen, showing a high signal central core with hypointense margin and no diffusion restriction.

Multiple small scattered pulmonary nodules.

Case Discussion

Biopsy was taken from the hepatic focal lesions revealed epithelioid hemangioendothelioma.

Epithelioid hemangioendothelioma (EHE) is a rare relatively low-intermediate grade malignant vascular tumor. It occurs around medium to large venous structures.

It can occur in a number of sites

In hepatic epithelioid hemangioendothelioma, focal lesions are usually hypoattenuating, showing marginal (target/halo pattern) contrast enhancement. They may show internal calcifications or cause capsular retraction. The lesions are usually marginally located.

In pulmonary epithelioid hemangioendothelioma, the pulmonary nodules are usually small and multiple, with random distribution as in this case. Calcifications are occasionally seen within the nodules.

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