Primary Cardiac Angiosarcoma

Abstract

Primary cardiac sarcoma is a rare clinical entity, with an incidence of 0.0001% in collected autopsy series. The majority of the literature describes a uniformly dismal prognosis with a median survival of only 6 months for these aggressive tumors. Standard surgery, adjuvant chemotherapy, and radiotherapy have been consistently unsuccessful. Early heart transplantation and novel radiation therapy approaches may offer a survival benefit in nonmetastatic tumors, but up to 80% of the patients present with systemic metastasis at diagnosis. Though several chemotherapeutic regimens have been tried, the role of chemotherapy is not well established and outcome data available is minimal. Liposomal doxorubicin (PLD) has been shown to be useful in the treatment of soft tissue sarcomas, and our case supports its use in cardiac angiosarcoma.