Metastatic epitheloid hemangioendothelioma (EHE): Role of systemic therapy and survival.

10079 Background: Data regarding the management of patients with metastatic EHE are almost nonexistent. Methods: From 1990 to 2010, 78 adult patients (pts) with a confirmed diagnosis of EHE were referred to one of the 9 participating institutions, and their medical records reviewed. Results: Forty-two (54%) patients had metastatic disease. Median age was 45 years. Systemic therapy was delivered in 34 cases (81%): single agent in 24 cases (71%). Twenty-two patients received cytotoxic chemotherapy: anthracycline-containing regimen (+/- ifosfamide): n=16; others: n=6. Twelve patients received an angiogenesis-targeting drug and/or a tyrosine kinase inhibitor: sorafenib n=6, thalidomide: n=2, metronomic cyclophosphamide: n=2, imatinib: n=2. No objective response was observed. Fourteen patients had stable disease > 6 months: cytotoxic chemotherapy n=8 (doxorubicin-containing regimen: n=4, other n=4); angiogenesis-targeting agent n=6 (sorafenib: n=3, metronomic cyclophosphamide: n=2, thalidomide n=1). 9/16 (56%)...