Primary Kaposi’s sarcoma of the heart in non-immunodeficient patient: case report and literature review

We present a case of Kaposi’s sarcoma (KS) of the heart in a 45-year-old non-immunodeficient woman with symptoms of pericardial effusion and cardiac tamponade. Computed Tomography (CT) coronary angiography and transesophageal echocardiography (TEE) showed a low-density tumorous mass (50 mm diameter) at the level of auricle of the right atrium spreading towards the superior vena cava, floating in the cavity of the right atrium. On histological examination, the tumor consisted of fibrovascular connective tissue with areas of necrosis and hemorrhage. Fibrous septae contained different sized thin walled capillary type blood vessels and lymphangioma-type vascular spaces. Vascular spaces were surrounded by extravasated erythrocytes, deposits of hemosiderin and sparse lymphoplasmacytoid infiltrates. On the periphery of tissue fragments and around vascular spaces, there was a cellular kaposiform proliferation of the spindled cells. Slit-like spaces between spindle cells contained erythrocytes. Nuclear pleomorphism of the spindled cells was minimal. Few mitotic figures were present. Spindle cells were Vimentin, CD34 and CD31 positive. More than 10 % of spindled cells were Ki67 positive. This characteristic histology and immunohistochemistry is consistent with Kaposi’s sarcoma. Patient has no history of other malignancies and no other primary tumor was detected. Patient also was negative for HIV infection. There are only 10 documented cases of primary Kaposi’s sarcoma of the heart in non-immunodeficient patients reported in the current medical literature. Our report is the first case in which imaging, histology and immunohistochemistry data are available.