Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with increasing incidence. However, its clinical characteristics remain unclear due to its low incidence. This study aimed to investigate the features of EHE. METHODS: Patients with EHE treated at our institution between January 2000 and June 2018 were enrolled. Data including demographic characteristics, treatment patterns, pathological results, and prognosis were collected, and a retrospective database was constructed for analysis. RESULTS: The cohort comprised 33 patients with a mean age of 48.0 ± 16.0 years. Eighteen (54.5%) patients were asymptomatic. The laboratory tests were unremarkable except in five and two patients who had increased CA 125 and CA 19-9, respectively. Twenty-one patients underwent surgery, while 12 patients underwent only biopsy. The postoperative morbidity rate was 28.6% (6/21). The anatomical sites of the primary lesions varied. Immunohistochemical staining was positive for CD34 and CD31 in most patients. Twenty-six patients (78.8%) were followed up at a range of 1–201 months, and 6 patients died during this period. The 1-, 3-, and 5-year cumulative survival rates were 96.2%, 87.0%, and 75.3%, respectively. The patients who had metastases or only underwent biopsy showed significantly higher mortality. CONCLUSIONS: EHE is a rare malignant vascular tumor that can occur in any site of the body. Surgery is the primary choice of treatment, and pathologic evaluation is the gold standard for diagnosis. Metastases and unresectability are associated with poor prognosis.