Primary Pericardial Angiosarcoma Complicated by Constrictive Pericarditis

Introduction

Secondary malignant cardiac tumors are metastases from primary tumors and 22 to 132 times more common than primary cardiac tumors (PCTs).^– PCTs are rare, with an estimated autopsy prevalence of 0.001-0.03%, and mostly (90%) benign.^,

Among PCTs, cardiac angiosarcoma is a rare subtype of soft tissue sarcoma and the most common primary malignant tumor of the heart. Angiosarcomas consist of irregularly shaped vascular channels lined by anaplastic epithelial cells with substantial areas of necrosis and hemorrhage. Cardiac angiosarcoma is an aggressive tumor with a high rate of local recurrence and systemic dissemination. Without surgical resection, it has a mean overall survival of 3.8 (SD 2.5) months. The rapid infiltrating growth of this tumor can lead to emergency clinical presentations including myocardial rupture, pericardial effusions, cardiac tamponade, and constrictive pericarditis (CP).

[…]