Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6119
Peer-review started: November 25, 2021
First decision: January 12, 2022
Revised: January 24, 2022
Accepted: April 22, 2022
Article in press: April 22, 2022
Published online: June 26, 2022
Processing time: 203 Days and 12.2 Hours
Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial cell tumor of the liver, consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. Immunohistochemistry is usually positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34. Hepatic EHE can have a varied clinical course; treatment includes liver transplantation, liver resection, chemotherapy, and radiation therapy.
A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography (CT) evaluation. An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells, containing intracellular vacuoles. Immunohistochemical staining found that the tumor cells were positive for CD34, CD31, and factor VIII-related antigen. The patient received four courses of combined chemotherapy and was followed-up for 13 years, at which time the patient was in stable condition without disease progression and a confined neoplasm, as evidenced by CT scans.
The histology and immunohistochemical characteristics of hepatic EHE are well described. Chemotherapy may be effective in patients with extrahepatic lesions.
Core Tip: The gold standard diagnosis for hepatic epithelioid hemangioendothelioma includes epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix, and positive vascular markers. Chemotherapy may be an effective treatment; close follow-up is necessary.