A rare cardiac neoplasm: case report of cardiac epithelioid angiosarcoma

Abstract

Primary cardiac angiosarcoma is a rare neoplasm and the epithelioid variant is exceedingly rare. We report a case of an epithelioid angiosarcoma that involved the right atrium and aorta of a 47-year-old male. The patient presented with atrial fibrillation and presyncopal spells. Following clinical evaluation, including computed tomography scan and trans-esophageal echocardiography, the neoplasm was surgically removed. It was a poorly differentiated malignant neoplasm composed of medium-sized epithelioid cells with a moderate amount of amphophilic cytoplasm. Immunohistochemical staining, including positive staining for CK22, AE1/AE3, melan-A, vimentin, and CD31, indicated the neoplasm was best categorized as an epithelioid angiosarcoma.