Clinical and radiological characteristics of Chinese patients with hepatic epithelioid hemangioendothelioma

BACKGROUND AND OBJECTIVES: To study clinical features, imaging findings, and prognosis of hepatic epithelioid hemangioendothelioma (HEHE) in Eastern Hepatobiliary Surgical Hospital and to improve the level of recognition and preoperative diagnosis of HEHE. DESIGN AND SETTINGS: This is a retrospective study. The study was conducted at Eastern Hepatobiliary Surgical Hospital in Shanghai, China from 1999 to 2012. METHODS: Clinical data of 15 pathology-confirmed HEHE patients admitted in our hospital from 1999–2010 were collected and analyzed retrospectively. RESULTS: Besides 7 cases of abdominal discomfort, this cohort of HEHE patients does not show common typical manifestations. Laboratory examinations found 8 low ALB/GLB cases, 3 mild anemia cases, and 1 high alanine aminotransferase case, but no abnormal α-fetoprotein, carcinoembryonic antigen, or CA19-9 cases. Plain computed tomography (CT) scanning found uneven multiple low-density lesions in most cases, dynamic enhanced CT scanning demonstrated peripheral enhancement in the hepatic arterial HA phase and more peripheral enhancement in the portal vein PV phase, and ring enhancement were detected in the delayed phase. Magnetic resonance imaging (MRI) demonstrated low signal intensity and even lower lesion centers in T1-weighted images, but slightly high signal intensity lesions and higher lesion centers in T2-weighted images. Enhanced MRI scanning showed ring enhancements but no obvious enhanced centers. Lesions were further enhanced in delayed MRI scanning. Prognosis of this cohort: 10 of these 15 patients were alive, including 4 cases with a tumor. CONCLUSION: Comprehending the clinical and radiological characteristics of HEHE facilitates the level of recognition and preoperative diagnosis of this disease, and promotes surgeons to take active and appropriate surgeries.