Angiosarcoma is a relatively uncommon primary malignant tumor of the heart derived from the endothelial cells. The authors report a case of primary cardiac angiosarcoma of the right atrium with pulmonary, hepatic, cerebellar and bony metastases in a 46-year-old man who clinically presented as pulmonary hemorrhage. The patient’s occupational history had occasional exposure to polyvinyl chloride. The autopsy examination of the cardiac angiosarcoma characterized macroscopically by a right atrial
tumor and histologically by anastomosing vascular channels and minute atypical pleomorphic endothelial cells. Immunohistological stainings were positive CD31, CD34 and focal positive factor VIII-related antigen. Clinical and pathologic features with briefly reviewed relevant literatures are discussed. This is the first reported description in the literature of a primary cardiac angiosarcoma with systemic metastases to multiple organs in Thailand.

Keywords: Angiosarcoma, Heart, Malignant, Metastases, Polyvinyl chloride