RESUMO
Primary cardiac tumors are rare, with a reported prevalence of 0.001 to 0.03% at autopsy series. Almost 75% of all primary tumors of the heart are benign, with primary malignant tumors accounting for the other 25%. Nearly half of the benign cardiac tumors are myxomas. Other primary tumors include papillary fibroelastoma, rhabdomyoma, fibroma, lipoma and hemangioma. In this review article, the clinical, pathologic and diagnostic feature of primary benign cardiac tumors is presented. In very rare occasions malignant tumors of the heart could be encounter such as primary sarcomas of the heart constituting less than 25% of primary cardiac tumors found in clinical practice. Cardiac sarcomas represent the commonest histology of primary malignant cardiac tumors, with angiosarcomas making up 33% of the cases. Sarcomas are common between the third and fifth decades of life and most frequently affect the right atrium.