Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular
tumor that most commonly presents within the liver. Patients with hepatic EHE are often candidates for
liver transplantation as the disease is usually multifocal at diagnosis. Although these patients achieve excellent early outcomes post-transplant, there are very few data regarding
tumor markers that can further direct
chemotherapy in hepatic EHE to prevent recurrent disease. The purpose of this study was to analyze the expression of the
angiogenic factor vascular endothelial growth factor (
VEGF) and its receptors in hepatic EHE. Six patients with hepatic EHE were assessed for
liver transplantation at our center. Pathology specimens of primary and recurrent EHE were analyzed by
hematoxylin and
eosin staining and by immunofluorescence for
VEGF,
fetal liver kinase 1 (Flk-1), and fms-related
tyrosine kinase 1 (Flt-1) expression. Five patients underwent
liver transplantation, and 1 patient underwent liver resection. Biopsy-proven recurrent EHE occurred in 3 patients.
VEGF expression was present in 100% of the EHE specimens examined, whereas Flt-1 expression was present in only 1 sample, and Flk-1 was not observed in any of the specimens. In 1 patient with recurrent hepatic EHE post-
liver transplantation, a progressive increase in the
VEGF fluorescence intensity and distribution was observed. In conclusion, in this series,
VEGF expression was observed in all hepatic EHE specimens analyzed. These data suggest that anti-
VEGF chemotherapeutic agents will be of use in patients with hepatic EHE, particularly as a means of reducing the
tumor volume prior to resection, as a means of treating unresectable or metastatic disease, or as an adjuvant
therapy in the setting of
liver transplantation.