Clinicopathologic Characteristics of Epithelioid Hemangioendothelioma in a Series of 34 Cases: a Double Center Retrospective Study |Biomedgrid

Objectives: Epithelioid hemangioendothelioma is an exceedingly rare vascular neoplasm with an indolent clinical course in various body sites. Its morphological appearance and prognosis are heterogeneous and complicated. Methods: We aimed to summarize the clinicopathologic features and prognostic factors of epithelioid hemangioendothelioma in retrospective research of 34 cases in two medical centers. Results: There were 14 (41.2%) males and 20 (58.8%) females, with a median age of 46.6 years (range 16-75 years). We compared the clinicopathological characteristics of lesions occurring from 7 body sites, including liver (N=15), lung (N=10), skin and soft tissue (N=3), bone (N=2), anterior mediastinum (N=2), adrenal gland (N=1) and left supraclavicular (N=1). In resected lesions, the multi-nodular growth pattern, the endovascular flower-like structure, and the forming of immature blood vessels were remarkable. Immunohistochemistry was essential for accurate diagnosis, especially in a biopsy. ERG and CD31 immunostaining are the most helpful markers. Ki67 index was mostly lower than 10%. Although there were multiple therapeutic options, patients who underwent operation had a better prognosis than unresectable groups in our study. Conclusions: Epithelioid hemangioendothelioma was clinically indolent, and the prognosis depended mainly on tumor location and the general state of the patients. Long-term follow-up should be applied to epithelioid hemangioendothelioma patients to improve clinical outcomes.