Rapid Development of Primary Right Atrial Angiosarcoma

Abstract

Cardiac angiosarcomas are rare and generally followed by a high level of metastasis with poor median survival outcomes. Echocardiograms, CT scans, and MRIs are the standard methods for finding sites of cardiac tumors; however, immunohistochemical confirmation is necessary for a definitive diagnosis of angiosarcoma. A 58-year-old male presented to the emergency room with one week of dyspnea on moderate exertion accompanied by chest pain and alleviated with rest. A workup done to evaluate mass found a single 5 x 3.5 x 4.8 cm mass heavily vascularized by the right coronary artery and left circumflex involving the free wall of the right atrium with no extension to the tricuspid valve. Surgical resection was performed, and immunohistochemistry was consistent with a primary cardiac angiosarcoma. An exudative fluid analysis on pericardial and pleural fluid analysis may warrant screening for malignancy more frequently in concurrence with a patient's history and presentation. Although the time from onset of symptoms to diagnosis of cardiac angiosarcoma is not well established, further investigation of such correlation may offer insight into survival post-treatment.

Keywords: cardiac angiosarcoma; cardiac tamponade; exudative fluid analysis; pericardial effusion; primary cardiac tumor; transesophageal echocardiogram.

Figure 1. Left heart catheterization depicting a right atrial mass (arrow) with vascularization through small branches coming from both the left circumflex artery and the right coronary artery, respectively.

Figure 2. TEE with right atrial mass (arrows) with hyperechoic core and hypoechoic filamentous fronds attached to the mass suggestive of significant vascularization.

Mass is closer to the SVC and thought to be either one large entity measuring up to 4.2 x 2.4 cm or two distinct adjacent 2 x 2 cm masses. TEE: transesophageal echocardiogram, SVC: superior vena cava.

Figure 3. MRI of the chest with a single 5 x 3.5 x 4.8 cm mass (arrows) involving the free wall of the right atrium.

No involvement of the tricuspid valve was found. MRI: magnetic resonance imaging.

Figure 4. H&E stain at 10x and 40x, respectively, depicting irregularly shaped anastomosing vascular channels lined by atypical endothelial cells which are polygonal-epithelioid in shape and mitotically active.

H&E: hematoxylin and eosin.

Figure 5. Immunohistochemical stain of biopsied mass is ERG positive, a marker for vascular tumors.

ERG: ETS-related gene, a vascular marker (ETS: erythrocyte transformation-specific transcription factor).