Primary cardiac leiomyosarcoma causing severe pulmonary hypertension

Suzuki, Ryusuke; Miyamoto, Tomoya; Hirayama, Ryo; Sakaguchi, Takeshi; Uekihara, Kenta; Yoshioka, Yuki

doi:10.1111/jocs.13241

J Card Surg

2017

DOI: 10.1111/jocs.13241

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Primary cardiac leiomyosarcoma causing severe pulmonary hypertension

Ryusuke Suzuki

Tomoya Miyamoto

Ryo Hirayama

et al.

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2018

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Cited by 8 publications

(9 citation statements)

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“…Cardiac sarcomas may involve all chambers of the heart, the mitral valve, the pericardium, present as metastatic lesions, and result in pulmonary hypertension . We present images of a primary synovial sarcoma of the interatrial septum presenting with cardioembolic stroke.…”

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Primary cardiac synovial sarcoma of the interatrial septum

et al. 2018

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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

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confidence: 97%

Primary cardiac synovial sarcoma of the interatrial septum

et al. 2018

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“…Cardiac sarcomas may involve all areas of the heart and great vessels including all the cardiac chambers, the valves, and the pulmonary artery . They may also present as metastases from other organs .…”

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confidence: 99%

“…Cardiac sarcomas may involve all areas of the heart and great vessels including all the cardiac chambers, the valves, and the pulmonary artery. [1][2][3][4][5][6] They may also present as metastases from other organs. 7 Primary intimal sarcomas arise from the intima of blood vessels and usually involve large blood vessels, but may also invade the myocardium.…”

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confidence: 99%

Intimal sarcoma of the left atrium

2018

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“…Intravenous tumors with extension into the right atrium (RA), right ventricle (RV), and the pulmonary artery (PA) may be benign or malignant. 1,2 They may present with lower extremity edema, dyspnea, and right-sided failure due to elevated pulmonary artery pressures. We report images of a patient with an intravenous leiomyoma extending to the PA who presented with recurrent syncope.…”

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Intravenous leiomyomatosis with extension to the pulmonary artery associated with syncope

et al. 2018

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Primary cardiac leiomyosarcoma causing severe pulmonary hypertension

Cited by 8 publications

References 6 publications

Primary cardiac synovial sarcoma of the interatrial septum

Primary cardiac synovial sarcoma of the interatrial septum

Intimal sarcoma of the left atrium

Intravenous leiomyomatosis with extension to the pulmonary artery associated with syncope

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