Cardiac angiosarcoma is a rare but lethal tumor that is difficult to diagnose and treat, due to its rapid local relapse and high incidence of systemic metastasis. The prognosis of cardiac angiosarcoma is dismal, with a mean life expectancy of only a few months. We herein report a case of unresectable angiosarcoma arising from the right atrium. The patient received first-line chemotherapy with weekly paclitaxel, and second-line therapy with vinorelbine and bevacizumab upon disease progression. The progression-free survival was 6 months and the overall survival was 7 months. The patient eventually succumbed to respiratory failure. A study of the present case and a review of the relevant literature suggest that treatment decisions for unresectable locally advanced or metastatic cardiac angiosarcoma are difficult, as the published literature on this disease mainly consists of case reports without sufficient data. Therefore, further clinical trials specific to the treatment of unresectable cardiac angiosarcoma are warranted.
Keywords: bevacizumab; cardiac angiosarcoma; paclitaxel; vinorelbine.