Primary cardiac synovial sarcoma of the interatrial septum

Abstract

Cardiac sarcomas may involve all chambers of the heart, 1-3 the mitral valve, 4 the pericardium, 5 present as metastatic lesions, 6 and result in pulmonary hypertension. 7 We present images of a primary synovial sarcoma of the interatrial septum presenting with cardioembolic stroke. A 32-year-old male presented with the sudden onset of left-sided hemiparesis and neglect. A head computed tomography (CT) scan revealed a proximal occlusion of the right middle cerebral artery, for which he underwent successful mechanical thrombectomy. Cardiac magnetic resonance imaging (MRI) revealed a 5.2 × 3.6-cm mass arising from the anterior portion of the interatrial septum (Figure 1). The electrocardiogram demonstrated a first-degree atrioventricular (AV) block. Positron emission tomography (PET) depicted the tumor without metastasis but revealed hypermetabolic lesions in both kidneys and the spleen.Ten days after the diagnosis, he underwent surgery to excise the mass.Cardiopulmonary bypass was initiated with ascending aortic and bicaval cannulation. Following arrest with antegrade, hypothermic crystalloid cardioplegia, the right atrium and interatrial septum were opened, allowing direct visualization of the tumor (Figure 2A). The entire mass was excised, after which a small defect in the subaortic curtain and the atrial septum were closed primarily ( Figure 2B). The patient was successfully weaned off cardiopulmonary bypass following a bypass time of 199 min and 146 min of cardioplegic arrest. An intraoperative transesophageal echocardiogram showed no residual tumor or valvular abnormalities. The first-degree AV block persisted but no pacemaker was required postoperatively. The patient had an uncomplicated postoperative course.Histopathologic examination revealed a biphasic malignant tumor with extensive necrosis and calcification (Figure 3). Additional molecular analysis revealed the translocation SS18:SSX, consistent with the diagnosis of synovial sarcoma. The oncology service did not recommend adjuvant therapy. One year later, the patient was doing well but still suffered from slight short-term memory impairment. Follow-up PET-CT scan and cardiac MRI showed no local recurrence and the hypermetabolic lesions in the kidneys and spleen had disappeared.FIGURE 1 Cardiac magnetic resonance imaging scan depicting a large mass arising from the anterior part of the interatrial septum (white arrows). A, The tumor has a rounded appearance and low signal intensity on T1-weighted spin echo images. B, On oblique short-axis cine gradient images, the appearance is more irregular with visible intracavitary components J Card Surg. 2018;33:391-392.wileyonlinelibrary.com/journal/jocs

Recurrent pulmonary artery intimal sarcoma with infiltration of the left coronary artery

“…Cardiac sarcomas may be primary or metastatic. They can involve the pulmonary artery, mitral valve, left atrium, pericardium, the septum, the right atrium, and ventricle, and may present with pulmonary hypertension or heart failure . We present images of the surgical management of a recurrent pulmonary artery sarcoma (PAS).…”

Synovial sarcoma of the aortopulmonary window

“…Myocardial sarcomas may involve all the chambers of the heart, the mitral valve, and the coronary and pulmonary arteries. [1][2][3][4][5] Synovial sarcomas can not only be found within the myocardium 6 but also in the pericardium. 7 We present images of a synovial sarcoma which presented as an aortopulmonary (AP) window mass, associated with pericardial tampo- (Figure 2A).…”

Angiosarcoma of the right atrium with extension to the superior and inferior vena cavae

“…Cardiac sarcomas may involve all the chambers and valves of the heart, and the coronary and pulmonary arteries, and can present with pulmonary hypertension, cardiac tamponade, and congestive heart failure . We present images of a patient with an angiosarcoma invading the right atrium (RA) and both cavae who presented with hemopytosis.…”