Diagnosis and treatment of hepatic epithelioid hemangioendothelioma

Objective To study the clinical features and treatment results of hepatic epithelioid hemangioendothelioma (HEHE). Methods A retrospective study was conducted on 26 patients with HEHE who were diagnosed and treated at the Eastern Hepatobiliary Surgical Hospital in Shanghai from November 2003 to December 2015. Results The main clinical manifestations of these 26 patients included abdominal pain (13), dizziness (1), anorexia (1), the other 11 patients were asymptomatic. Ultrasound test revealed mixed echogenicity in 17 patients (17/25, 68.0%). CT study showed hypointensity lesions in 10 patients (10/12, 83.3%). MRI scans identified T1WI hypointense signals in 12 patients (12/14, 85.7%) and T2WI hyperintense signals in 11 patients (11/14, 78.6%). Platelet distribution width (PDW) assay indicated 18 patients <15.5 fl (18/22, 81.8%), P<0.05. all the patients performed tumor makers test, including AFP, CEA, CA19-9. Slightly elevation of CA19-9 was merely identified in one patient. All the patients were confirmed CD34 positive through immunohistochemistry stains. The 1, 3, 5-year survival rates of these patients were 87.7%, 57.0%, 43.4%, respectively. Median survival was 61 months. Conclusions HEHE should be considered based on the suggestive imaging findings for the patients with negative tumor markers after exclusion of hemangioma. PDW assay may have a key role in HEHE diagnosis. Key words: Liver neoplasms; Epithelioid hemangioendothelioma; Platelet distribution width