Fact-sheet: Epithelioid hemangioendothelioma of the liver
Updated on 03/27/2020 at 3:35 PM
View all RADEOS cases associated with this fact-sheet
Definition
- Rare mesenchymal tumor of the liver with malignant potential intermediate between hemangioma and Angiosarcoma
- Tumor of medium to large veins, composed of round to spindle-shaped endothelial cells projecting into the vascular space
- Preferentially affects young women
- Sometimes metastatic at diagnosis: lungs, lymph nodes, bone
Clinical features
Nonspecific clinical presentation: abdominal pain, jaundice, hepatomegaly
Known risk factors: vinyl chloride exposure and certain oral contraceptives
Laboratory findings
Nonspecific abnormalities on liver function tests
Ultrasound
Nonspecific heterogeneous, hypoechoic lesion(s)
CT
- Peripheral location with capsular retraction, a suggestive feature
- Central calcifications possible and suggestive
- Atrophy of the tumor-bearing liver/hypertrophy of the non-tumor-bearing liver
- After injection, peripheral enhancement of the hypodense lesion giving a target or bull's-eye appearance
- Isodense on delayed phase
- Sometimes a hepatic or portal vein tapering and terminating at the periphery of the tumor: suggestive "lollipop sign"
MRI
- Peripheral location with capsular retraction, a suggestive feature
- T1 hypointensity, heterogeneous T2 hyperintensity
- Target appearance on T2
- More intense enhancement of healthy parenchyma than of the lesions
- Sometimes a hepatic or portal vein tapering and terminating at the periphery of the tumor: "lollipop sign"
Management
Radiologic follow-up, surgical resection, or liver transplantation depending on symptoms and rate of progression
Differential diagnosis
- Angiosarcoma
- Cholangiocarcinoma
- Metastases