Hepatic epithelioid hemangioendothelioma after thirteen years'follow-up:A case report and review of literature

BACKGROUND 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.CASE SUMMARY 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.CONCLUSION The histology and immunohistochemical characteristics of hepatic EHE are well described.Chemotherapy may be effective in patients with extrahepatic lesions.