BACKGROUND: Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges. The aim of the present study was to determine the treatment and prognosis of this entity.

DATA SOURCES: A systematic literature search was conducted using PubMed, Embase and Chinese Biomedical Literature database, to identify articles published from January 1980 to July 2017. Search terms were "hepatic angiosarcoma" and "liver angiosarcoma". Additional articles were retrieved through manual search of bibliographies of the relevant articles. Pooled individual data concerning the prognosis following various therapeutic modalities were analyzed.

RESULTS: A total of 75 articles involving 186 patients were eligible for inclusion. The median overall survival (OS) was 8 months, with 1-, 3-, and 5-year OS rates of 36.6%, 22.3%, and 12.0%, respectively. The median OS after partial hepatectomy (n?=?86), chemotherapy (n?=?36), liver transplantation (n?=?17), and supportive care (n?=?46) were 15, 10, 5 and 1.3 months, respectively. Small tumor size (<10?cm) was the only significant favorable factor for OS after partial hepatectomy (P?=?0.012).

CONCLUSIONS: Despite the dismal prognosis, partial hepatectomy could prolong the survival of hepatic angiosarcoma patients, particularly those with tumors <10?cm. Chemotherapy could be an option for unresectable disease. Liver transplantation is not a recommendable option for the management of this malignancy.