Abstract
BACKGROUND: Primary tumors of the heart are a rare phenomenon. Lymphatic malformations are congenital anomalies of the lymphatic system that tend to grow progressively. Lymphatic malformations are typically found in the cervical and axillary regions and found on pediatric patients. We report a 40-year-old woman with giant epimyocardial lymphatic malformation. CASE PRESENTATION: A 40-year-old woman was assessed due to suspected traumatic cardiac tamponade. Computed tomography of the heart and cardiac magnetic resonance imaging were compatible with either a large pericardial hemangioma or angiosarcoma. The tumor infiltrated deeply into the myocardium and could only be partially resected. Histopathological diagnosis was a cardiac lymphatic malformation with micro- and macrocystic components. The patient has remained asymptomatic for fourteen years after the surgery. In the latest follow-up, her left ventricular function had remained normal and the maximum thickness of the residual tumor had regressed. CONCLUSIONS: Even when a complete removal of a cardiac lymphatic malformation is not possible, a debulking procedure can yield a good long-term result.