Section: Hepatobiliary Sub-section: Liver Curriculum: Curriculum, page 85
Definition
- Neoplasms of vascular origin
- Arise in soft tissue, bones and visceral organs (mostly lung and liver)
- Hepatic EHE develops from the endothelial cells lining the sinusoids and progresses along the sinusoids and vascular pedicles
- Rare
- No known causative factor
Diagnosis
- Tumor markers normal
- On imaging studies, the lesions are frequently confused with cholangiocarcinoma, metastatic carcinoma, sclerosing haemangioma or inflammatory pseudotumours
- Hypoechoic or heterogeneous on US, hypodense on CT with peripheral and/or central marginal enhancement on the arterial phase becoming isodense during the later phase and may display a halo or target pattern of enhancement
- Histologic examination shows a tumour composed of epithelioid and dendritic cells in variable proportions with a propensity for invasion of hepatic and portal veins, an overall ill-defined growth pattern and infiltrative margins.
- Immunostaining for factor VIII-related antigens is highly specific for EHE, and most tumours also stain positive for CD34 and CD31 endothelial markers.
Treatment
- No consensus
- Resection if possible
- Transplant an option