Surgical management of large undifferentiated cardiac sarcoma involving the right ventricle to pulmonary trunk

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Surgical management of large undifferentiated cardiac sarcoma involving the right ventricle to pulmonary trunk Hiroshi Furukawa1 · Takeshi Honda1 · Takahiko Yamasawa1 · Yuji Kanaoka1 · Kazuo Tanemoto1 Received: 23 May 2019 / Accepted: 9 September 2019 © The Japanese Association for Thoracic Surgery 2019

Abstract A large intra-cardiac tumor from the right ventricle to pulmonary trunk was detected by contrast-enhanced computed tomography and transthoracic echocardiography in a 59-year-old woman with progressive dyspnea on effort and acute congestive right-sided heart failure. Emergent surgical management was performed with cardiopulmonary bypass under cardiac arrest, however, tumor resection was incomplete, because it originated from the interventricular septum. Concomitant tricuspid valve replacement using a bioprosthesis was required due to the involvement of the septal leaflet of the tricuspid valve. Although large pulmonary thromboembolism was initially suspected, a pathological examination confirmed undifferentiated cardiac sarcoma. The patient did not consent to additional neoadjuvant chemotherapy or radiation therapy. After palliative surgical management, she was discharged. Recurrence rapidly progressed and the patient died approximately 2 months after surgery. We herein present a successful palliative surgical case of large cardiac undifferentiated sarcoma originating from the intraventricular septum and involving the right ventricle to pulmonary trunk. Keywords  Cardiac sarcoma · Undifferentiated sarcoma · Congestive heart failure · Tricuspid valve replacement · Right ventricle

Introduction

Case

Primary cardiac sarcoma is an extremely rare phenomenon that is only reported in between 0.001 and 0.03% of the general population [1]. Most cases of cardiac sarcoma are clinically recognized as inoperative, and conservative medical treatment has been conducted due to its poor prognosis; the surgical outcome of primary cardiac sarcoma has an approximately 20% survival rate 1 year after its diagnosis and treatment [2]. Therefore, surgical strategies and indications remain controversial. We herein demonstrate successful palliative surgical management for a complex case of large cardiac sarcoma originating from the interventricular septum (IVS) and involving the right ventricle (RV) to pulmonary artery (PA) trunk.

A 59-year-old woman with no specific medical history had dyspnea on effort for 1 year. She was recently referred to our hospital with the initial symptoms of progressive dyspnea on effort and orthopnea with worsening oxygenation. Chest X-ray showed pulmonary congestion with cardiomegaly (Fig. 1), and acute congestive heart failure was diagnosed. Contrast-enhanced computed tomography revealed a large intra-cardiac mass tightly occupying the space from the RV to PA trunk (Fig. 2). Transthoracic echocardiography showed a large intra-cardiac tumor from the RV to PA trunk and compression of the left ventricle with a typical D-shape. She had no specific medical history or lifestyle, and pulmonary