Clinical Experience with Primary Hepatic Epithelioid Hemangioendothelioma: Retrospective Study of 33 Patients

Background This multicenter-based retrospective study aimed to investigate the prognostic factors and report our experiences with the diagnosis and treatment of hepatic epithelioid hemangioendothelioma (HEHE), a rare malignant vascular tumor. Methods A total of 33 patients with HEHE from two centers between 2004 and 2011 were retrospectively reviewed with respect to their clinical, radiologic, and pathologic characteristics; treatment modalities and outcomes; and potential prognostic factors. Results A total of 17 patients underwent liver resections (LRs) alone, 12 patients had transcatheter arterial chemoembolization (TACE) alone, three patients had LR followed by TACE, and one patient underwent liver transplantation (LT). The difference of overall survival (OS) between LR and TACE was not significant ( p = 0.499). Older patients [≥47 years, n = 17; p = 0.035, hazard ratio (HR) = 7.0), those with symptoms ( n = 17; p = 0.001, HR = 86.5], and those with an elevated serum CA19-9 level (>37 U/ml, n = 5; p = 0.018, HR = 5.0) had a poorer OS, according to univariate analysis. The presence of symptoms was validated as a prognostic factor ( p = 0.012) by multivariate analysis. Conclusions Liver resection and TACE have comparable outcomes in HEHE patients. The presence of symptoms indicates a poor prognosis. Older age and elevated serum CA19-9 are potential negative impact factors on outcome.