Primary cardiac synovial sarcoma of the interatrial ...

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analysis revealed the translocation SS18:SSX, consistent with the diagnosis of synovial sarcoma. The oncology service did not recommend adjuvant therapy.

DOI: 10.1111/jocs.13733

IMAGES IN CARDIAC SURGERY

Primary cardiac synovial sarcoma of the interatrial septum Nicolas De Hous BM1

|

Bernard Paelinck MD, PhD2 | Jan Van den Brande MD3 |

Vasiliki Siozopoulou MD4 | Steven Laga MD1 1 Department

of Cardiac Surgery, University Hospital of Antwerp, Edegem, Antwerp, Belgium

2 Department

of Cardiology, University Hospital of Antwerp, Edegem, Antwerp, Belgium

3 Department

of Oncology, University Hospital of Antwerp, Edegem, Antwerp, Belgium

4 Department

of Pathology, University Hospital of Antwerp, Edegem, Antwerp, Belgium

Correspondence Nicolas De Hous BM, Department of Cardiac Surgery, University Hospital of Antwerp, Wilrijkstraat 10, 2650 Edegem, Antwerp, Belgium. Email: [email protected]

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. J Card Surg. 2018;1–2.

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

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FIGURE 3 Histopathologic examination of the resected tumor (hematoxylin-eosin stain, ×200). The vital tumor tissue had a biphasic morphology: an epithelial component forming gland-like structures (black arrows) and a stromal component with myxoid changes and spindle cells (white arrows) REFERENCES

FIGURE 2 A, Perioperative picture after opening the right surface of the interatrial septum, with visible cutting edges (black arrows). The tumor was enveloped in the interatrial septum by atrial myocardium (white arrow). B, Resected tumor, about 5.5 × 3.5 cm. One segment penetrated into the left atrial cavity and very likely constituted the source of embolism (black arrow). Part of the tumor was anchored to the subaortic curtain (white arrow), of which a small part had to be resected along with the tumor

1. Rao JN, Gowda D, Amand R, Desai NB. Angiosarcoma of the right atrium and right ventricle. J Card Surg. 2017;32:807–808. 2. Dolenc J, Jelenc M, Dimitrovska L, et al. Cardiac autotransplantation and extracellular matrix patch reconstruction for a left atrial sarcoma. J Card Surg. 2017;32:95–96. 3. Pieraets MW, Soliman MAH, von Straten BHM. Intimal sarcoma of the left atrium. J Card Surg. 2018;33:179–180. 4. Aguilar C, Soca R, Guillen M, et al. Cardiac undifferentiated pleiomorphic sarcoma incidentally diagnosed during mitral valve replacement. J Card Surg. 2017;32:91–92. 5. Yu L, Gu T. Giant pericardial synovial sarcoma. J Card Surg. 2017;32:266–267. 6. Manuel V, Dinato FJ, Gutierrez PS, et al. Cardiac metastatic endometrial stromal sarcoma 17 years after hysterectomy. J Card Surg. 2017;32:636–638. 7. Suzuki R, Miyamoto T, Hirayama R, et al. Primary cardiac leiomyosarcoma causing severe pulmonary hypertension. J Card Surg. 2017;32:794–796.

CONFLICTS OF INTEREST The authors have no conflicts of interest to disclose.

How to cite this article: De Hous N, Paelinck B, Van den Brande J, Siozopoulou V, Laga S. Primary cardiac synovial sarcoma of the interatrial septum. J Card Surg. 2018;1–2.

ORCID Nicolas De Hous

https://doi.org/10.1111/jocs.13733 http://orcid.org/0000-0001-9965-0916

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