Clinicopathological and cytogenetic features of hepatic angiosarcoma and epithelioid hemangioendothelioma

Background and Objectives: Hepatic angiosarcoma (HAS) and epithelioid hemangioendothelioma (HEHE), are rare liver malignancies. This study aimed to evaluate prognostic factors, including erythroblast transformation-specific-related gene (ERG) expression, in HAS and HEHE. Methods: Twenty-four HAS patients and 38 HEHE patients were retrospectively enrolled between January 1986 and June 2014. Immunohistochemistry was performed to examine expression of CD31, CD34, factor VIII-related antigen and ERG, and fluorescence in situ hybridization was used to determine expression and rearrangement of ERG. Results: HAS patients had a significantly shorter mean overall survival than HEHE patients (P < 0.05). Multivariate analysis showed that presence of extrahepatic me. tastases (P = 0.04) and histological necrosis (P = 0.02) were independent unfavorable prognostic factors for HAS, and presence of extrahepatic metastases (P < 0.01), advanced mitosis grading (P < 0.01), and high Ki-67 proliferation index (P < 0.01) were independent unfavorable prognostic factors for HEHE. ERG expression without any gene rearrangement was identified in HAS and HEHE. Conclusions: Presence of extrahepatic metastases and advanced mitosis grading are independent unfavorable prognostic factors for HAS and HEHE, with ERG protein as a highly sensitive biomarker.