Pericardial PEComa: echocardiographic features.

The patient was a 19 year old man referred to our center due to progressive effort dyspnea and left sided pleuritic chest pain for 1 month. Echocardiographic examination showed a posteriorly located, giant, well demarcated mass, that compresses the left atrium. It was encased by moderate pericardial effusion. Histhopathological analysis disclosed neoplastic cells arranged around blood vessels with immunoreactivity for HMB-45 indicative of PEComa (Perivascular Epitheloid Cell differentiation). Six month follow up was uneventful. Up to the best of our knowledge, this is the first case of pericardial PEComa reported in the literature.