Recurrent Primary Cardiac Sarcoma Managed With Radical Cardiac ...

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CASE REPORT XIAO ET AL RESECTION FOR RECURRENT CARDIAC SARCOMA

Ann Thorac Surg 2015;100:728–30

Recurrent Primary Cardiac Sarcoma Managed With Radical Cardiac Resection and Pneumonectomy Kevin Xiao, Keith B. Allen, MD, A. Michael Borkon, MD, Sanjeev Aggarwal, MD, J. Russell Davis, MD, Jim Stewart, MD, Alex Pak, MD, and R. Scott Stuart, MD University of Missouri–Kansas City School of Medicine and Department of Cardiothoracic/Vascular Surgery, St. Luke’s Mid America Heart & Vascular Institute, Kansas City, Missouri

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Primary cardiac tumors are extremely rare. Although complete surgical resection of malignant primary cardiac tumors results in an improved survival compared with no intervention, the overall prognosis is generally poor, with treatable recurrent primary cardiac sarcomas being extremely rare. We report a patient with a recurrent primary cardiac sarcoma obstructing the left atrium managed with radical cardiac resection, including right pneumonectomy, with 21-month postprocedural survival. (Ann Thorac Surg 2015;100:728–30) Ó 2015 by The Society of Thoracic Surgeons

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rimary cardiac tumors are extremely rare, with an incidence of less than 0.03% in autopsy series [1, 2]. Although 75% of primary cardiac tumors are benign, 25% are highly malignant, with sarcomas being the most common [1–3]. Complete resection of malignant cardiac tumors results in an improved survival of 6 to 12 months compared with a median survival of 1 to 2 months without resection [4–6]. Considering the overall dismal prognosis, treatable recurrent primary cardiac sarcomas are extremely rare. We report a patient with a recurrent primary cardiac sarcoma obstructing the left atrium managed with radical cardiac resection, including right pneumonectomy, with 21-month postprocedure survival.

Fig 1. Computed tomography shows large recurrent left atrial mass prolapsing through the mitral valve (X) into the left ventricle.

offered reoperation with plans for complete resection. Through a redo sternotomy, cardiopulmonary bypass was initiated using bicaval cannulation. Dissection of the hilum of the right lung showed that the mass could be completely resected using a right pneumonectomy and left and right atrial resections with reconstruction. Cardioplegic arrest was initiated and the right atrium opened, which demonstrated tumor within the septum and extending toward the superior and inferior vena cava. The left atrium was entered through the septum, and the large prolapsing mass was withdrawn back across the mitral valve and into the left atrium. The mass involved the inferior and superior right pulmonary veins. The mitral and tricuspid valves were not involved with tumor. Both caval junctions were divided, and the right side of the left atrium was resected. The right pulmonary artery

A 52-year-old man presented with New York Heart Association Functional Class IV heart failure with worsening dyspnea and new-onset atrial fibrillation. His history included complete resection of a 4-cm malignant primary left atrial sarcoma treated with postoperative chemotherapy 2 years earlier. An echocardiogram showed a mobile mass in the left atrium with a 7-cm 4-cm portion of the tumor prolapsing across the mitral valve (Fig 1). Computed tomography of the chest showed the mass involved the confluence of the superior and inferior right pulmonary veins with extension into the right atrium. Positron emission tomography showed no evidence of extra thoracic disease and no mediastinal adenopathy. Considering the patient’s age, good functional status, and no extrathoracic evidence of tumor, the patient was Accepted for publication Sept 29, 2014. Address correspondence to Dr Allen, 4320 Wornall Rd, Medical Plaza II, Ste 50, Kansas City, MO 64111; e-mail: [email protected].

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

Fig 2. The right atrium is reconstructed using a pericardial patch with preservation of the tricuspid valve (TV). The left atrial reconstruction, already completed, is out of view, with only a small portion of the patch visible in the photograph. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.09.087



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Fig 3. The superior and inferior vena cavae are bridged with a 26-mm Hemashield (Maquet, Wayne, NJ) graft. (A) An eye cautery creates a generous oval in the Hemashield graft (B) to complete the anastomosis (X) to the neoright atrium.

tricuspid and mitral valves. The anastomosis from the cava graft to the right atrial patch was patulous, with good flow. Histologic analysis showed a high-grade sarcoma consisting mainly of undifferentiated malignant cells. Focal tumor necrosis was present, and there was a high mitotic rate of up to 10 mitotic figures per high-power field. Surgical margins were clean, and the level 4R, 7, 8, 9, 10, and 11 lymph nodes were negative for tumor. The patient recovered uneventfully, with discharge to home on postoperative day 10 (Fig 4B). He received four cycles of adjunctive chemotherapy using a combination of doxorubicin and ifosfamide. Transthoracic echocardiograms at 3, 6, 12, and 18 months demonstrated no evidence of local recurrence, with no heart failure symptoms. Unfortunately, evidence of extrathoracic metastatic disease developed and the patient died 21 months after his operation.

Comment Primary malignant cardiac neoplasms are extremely rare. Of these, angiosarcoma is the most common of the malignant tumors, followed by undifferentiated sarcoma [1, 6] Mean age of presentation varies between 40 and 50 years, but generally before the sixth decade of life [1, 2]. Common Fig 4. (A) Intraoperative photograph demonstrates the completed en block resection of the recurrent left atrial sarcoma with reconstruction of the left and right atrium and the pneumonectomy space (P). (B) Discharge chest roentgenogram shows almost complete filling of the pneumonectomy space, without mediastinal shift.

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was transected with an endostapler (Ethicon, Somerville, NJ), followed by stapled (Ethicon) division of the right mainstem bronchus that allowed en bloc removal of the entire mass. Level 4R, 7, 8, and 9 lymph nodes were resected for staging. Cardiac reconstruction followed, with creation of a neoleft atrium out of bovine pericardium while maintaining left pulmonary venous return and a competent mitral valve. Right atrial reconstruction was performed using an oval pericardial patch and maintaining coronary sinus drainage into the neoright atrium (Fig 2). Bridging the superior and inferior vena cava was accomplished with a 26-mm Hemashield (Maquet, Wayne, NJ) tube graft (Fig 3A). An oval was resected from the side of the Hemashield graft, which was then anastomosed to an opening created in the right atrial patch to complete the cardiac reconstruction (Fig 3B). Two Myodex Bipolar, Steroid-eluting Epicardial Pacing Leads (St. Jude Medical, Minneapolis, MN) were attached to the right ventricle, tunneled to a subcutaneous infracostal pocket, and attached to a single-chamber pacemaker (St. Jude Medical). The patient was weaned off cardiopulmonary bypass uneventfully with a paced rate of 90 (Fig 4A). Intraoperative transesophageal echocardiography demonstrated excellent right and left ventricular function with competent

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presenting symptoms are often nonspecific, including dyspnea, pain, or shortness of breath [4]. Echocardiography is the preferred diagnostic study, as in our patient, with metastatic workup to include positron emission tomography and computed tomography [2, 7]. Owing to the aggressive nature of these tumors, metastasis is present in 80% of the patients at presentation [2]. In the absence of metastatic disease, complete surgical resection of the tumor is the mainstay of treatment and is linked with a significantly longer median survival of 12 months compared with 1 month for patients who do not undergo resection [1]. Included among published techniques to allow complete tumor resection are explant of the heart with back table tumor resection and reconstruction [4]. Heart transplant may also be considered in select patients; however, scarcity of donor hearts and the generally short survival, even with complete tumor removal, has limited this option to a few case studies [4, 8]. The role of adjuvant chemotherapy and radiotherapy before or after surgical resection is undetermined due to the scarcity of cases. Although adjuvant therapy is frequently incorporated into care algorithms, whether survival is improved, particularly if complete resection of the tumor was not accomplished, remains to be determined [1, 4, 9]. The 21-month survival in our patient was significantly longer than the median 12-month survival typically observed after resection. The histologic analysis showed our patient had a highly malignant tumor; however, despite its recurrent nature, we were able to


completely resect it, without evidence of extracardiac or lymphatic invasion on the final pathologic analysis. In conclusion, recurrent primary cardiac sarcomas that can be managed successfully with complete repeat resection are extremely rare. Aggressive surgical resection should be offered to patients with reasonable functional status in whom complete resection can be obtained.

References 1. Hamidi M, Moody JS, Weigel TL, Kozak KR. Primary cardiac sarcoma. Ann Thorac Surg 2010;90:176–81. 2. Odim J, Reehal V, Laks H, Mehta U, Fishbein M. Surgical pathology of cardiac tumors. Two decades at an urban institution. Cardiovasc Pathol 2003;12:267–70. 3. Silverman NA. Primary cardiac tumors. Ann Surg 1980;191: 127–38. 4. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther 2008;6: 1217–22. 5. Kim PM, Correa AM, Blackmon S, et al. Outcomes after rightsided heart sarcoma resection. Ann Thorac Surg 2011;91:770–6. 6. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 2006;69:387–95. 7. Meng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol 2002;84:69–75. 8. Gowdamarajan A, Michler RE. Therapy for primary cardiac tumors: is there a role for heart transplantations? Curr Opin Cardiol 2000;15:121–5. 9. Llombart-Cussac A, Pivot X, Contesso G, et al. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer 1998;78:1624–8.