Primary Right Atrial Cardiac Angiosarcoma in Patient with Poland Syndrome: Case Report and Review of the Literature

Poland syndrome is a rare congenital disorder presenting with an absence of unilateral large pectoral muscle and other malformations of the anterior chest wall and breast. Although there are reports of associated malignancies, including leukemia, non-Hodgkin lymphoma in Poland syndrome, solid tumors are rarely seen. Primary cardiac angiosarcoma is a rare malignant cardiac neoplasm with early metastasis and poor prognosis. In this case report, we present a case of primary cardiac angiosarcoma in a 24-year-old woman with Poland syndrome who admitted with cardiac tamponade.

A 24-year-old woman presented to the emergency room with severe shortness of breath and chest pain for two days. The patient had a history of Poland syndrome and an artificial left breast prothesis operation 3 years ago. Echocardiographic findings were compatible with cardiac tamponade. Patient moved to the coronary intensive unit and urgent pericardiocentesis was performed through apical approach. FDG- PET/CT showed that the right atrium mass was 44x48 mm in size, no systemic metastases were detected at the time. Cardiac Magnetic Resonance Imaging (MRI) has been performed to gain a better imaging of the tumor anatomy and morphology. Because of the aggressive enlargement of the tumor, immediate operation was planned. She was discharged after successful uneventful follow-up.

Primary cardiac angiosarcoma is a rare malignant cardiac neoplasm with early metastasis and poor prognosis. Since cardiac angiosarcomas have aggressive course and poor prognosis, patients with Poland syndrome should be followed on regular bases.