Primary angiosarcoma of the heart.

Primary hemangioendotheliosarcoma is the least common form of sarcoma of the heart, itself an uncommon tumor. A review of most of the reported cases is presented. This study revealed that although the syndrome produced by cardiac sarcoma point to a strikingly characteristic picture, the diagnosis is almost always confused, especially with pericardial inflammatory diseases, and delayed. Successful treatment generally has not been available. This is the only case we know of that has been treated by resection utilizing cardiopulmonary bypass. The appearance of metastases in unusual locations in our patient, despite total excision of the primary, suggests tumor embolization during the bypass and the need for intra-operative chemotherapy, as well as such treatment before or after surgery.