Presentation
Elevated liver enzymes. Mass on liver ultrasound.
Patient Data
Contrast-enhanced axial CT of the abdomen and pelvis.
The arterial phase shows hepatomegaly with ill-defined enhancement involving a large portion of the right hepatic lobe. A focal area of hypo enhancement with overlying capsular retraction is present lateral to the gall bladder fossa.
On the portal venous phase, the focal area of decreased density and capsular retraction remains evident while the remainder of the liver is generally homogeneous appearing.
There is no adenopathy, ascites or other extra-hepatic abnormalities.
MRI liver with and without contrast shows increased T2 signal intensity throughout a large portion of the right hepatic lobe.
Liver, right lobe #1, biopsy: Consistent with epithelioid hemangioendothelioma.
Liver, right lobe #2, biopsy: Consistent with epithelioid hemangioendothelioma.
Liver, left lobe, biopsy: Rare atypical cells.
FDG PET-CT shows abnormal moderate FDG avidity of the right hepatic lobe.
There is concurrent mild, homogeneous FDG avidity in the axial and proximal appendicular skeleton likely representing marrow hyperplasia.
Case Discussion
Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy that is locally invasive but may also metastasize. It most commonly affects the liver followed by the lungs and bones.
For hepatic EHE (as in this case), a study of 60 lesions showed that two-thirds will demonstrate increased FDG uptake, and one-third may have uptake equal to the surrounding uninvolved liver.