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J Thorac Cardiovasc Surg 2007;133:1667-1669
© 2007 The American Association for Thoracic Surgery

Brief Communication

Primary Ewing sarcoma invading the heart: Resection and reconstruction

Subroto Paul, MDa, Tharumenthiran Ramanathan, MD, PhD, FRACSb, Daniel M. Cohen, MDa, Abraham Lebenthal, MDa, Lambros Zellos, MDa, Sary F. Aranki, MDb, David J. Sugarbaker, MDa,*

a Division of Thoracic Surgery, Department of Surgery, Brigham and Women’s Hospital, Boston, Mass
b Division of Cardiac Surgery, Department of Surgery, Brigham and Women’s Hospital, Boston, Mass.

Received for publication January 6, 2007; accepted for publication February 7, 2007.

* Address for reprints: David J. Sugarbaker, MD, Brigham and Women’s Hospital, Division of Thoracic Surgery, 75 Francis St, Boston, MA 02115. (Email: dsugarbaker{at}partners.org).

Clinical Summary

We describe the case history of a 14-year-old boy who came to his pediatrician with a cough. Chest radiograph revealed a large mediastinal mass. Workup with chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed that the mass had invaded the right pulmonary parenchyma, both atria, and the superior vena cava (SVC) (Figure 1, A). CT-guided biopsy revealed the tumor to be Ewing sarcoma/primitive neuroectodermal tumor. The patient underwent sarcoma-based chemotherapy with 18 weeks of vincristine, ifosafamide, doxorubicin (Adriamycin), and etoposide followed by 2 additional weeks of vincristine, doxorubicin, and isofamide. Repeat imaging revealed the mass to be downsized by chemotherapy (Figure 1, B). Bone scan, abdominal CT, and positron emission tomography–CT did not reveal any metastases. The patient was referred for surgery. Preoperative workup included pulmonary function tests (forced expiratory volume in 1 second = 2.59 [67%], forced vital capacity = 3.00 [67%]), a quantitative ventilation/perfusion scan (quantitative perfusion [left lung] = 81%; quantitative perfusion [right lung] = 19%), and an echocardiogram (ejection fraction = 65% with normal left and right ventricular function).


Figure 1
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  		Figure 1. A, Chest CT demonstrating extensive mediastinal mass involving right and left atria. B. Chest MRI after chemotherapy showing significant reduction in tumor volume.

 
A median sternotomy was performed and both pleurae and the pericardium were entered. The mass involved the distal third of the SVC, the free wall of the right and left atria, the interatrial septum, and the right lung, but was deemed resectable. The patient was placed on cardiopulmonary bypass via aortic and bicaval cannulation and cooled to 28°C with the heart arrested with antegrade blood cardioplegia. An en bloc resection of the lower third of the SVC, the free wall of the right and left atria along with the interatrial septum, and a right pneumonectomy were performed (Figure 2, A). Frozen section margins were negative for tumor involvement. The interatrial septum and free wall of the right atrium were reconstructed with glutaraldehyde-treated pericardium (Figure 2, B and C). The SVC was reconstructed with bovine pericardium fashioned into a tube using a double row of staples (US Surgical Corporation, Norwalk, Conn) (Figure 2, D). The distal end of the graft was anastomosed in an end-to-end fashion to the SVC. The proximal end of the tube graft was anastomosed to a transverse opening in the right atrial pericardial patch. The pericardium was reconstructed with porcine submucosal tissue grafts (Cormatrix; Cormatrix Cardiovascular, Atlanta, Ga) to prevent cardiac herniation and to provide support to the SVC graft. The patient was weaned from bypass and the chest closed. The patient had an uneventful postoperative course and was discharged from the hospital on postoperative day 14 with a regular atrial rhythm. Final pathologic examination confirmed negative resection margins with only 40% viable tumor. The patient received further adjuvant chemoradiation therapy 6 weeks postoperatively.


Figure 2
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  		Figure 2. A, Gross pathology of mass. B, Operative photograph of left and right atrial remnants (left atrium and right atrium, respectively). C, Operative photograph of reconstructed left atrium with right atrial reconstruction ensuing. D, Operative photograph of reconstructed SVC. LA, Left atrium; RA, right atrium.

 
Discussion

The Ewing sarcoma family of tumors, which includes Ewing sarcoma, primitive neuroectodermal tumor, neuroepithelioma, and Askin tumor, is thought to derive from neural crest cells. Genetic analysis of these tumors has revealed a translocation between chromosomes 11 and 22 that is shared by this family of sarcomas. Although typically arising from the bony skeleton, these tumors have been described to appear in almost any soft tissue in the body.1Go

Our case illustrates the importance of multimodality treatment in dealing with Ewing sarcoma. This patient’s tumor was unresectable at initial presentation, and chemotherapy was essential in downsizing the tumor to allow resection. Furthermore, resection alone is associated with an 80% to 90% chance of metastatic disease inasmuch as these tumors are highly aggressive and subclinical metastasis is assumed.1Go Hence, chemotherapy up front not only facilitates surgical resection but also improves long-term outcomes.1Go

This patient’s mediastinal tumor with cardiac invasion has been reported only once before in the English literature. There have been several reports of these tumors metastasizing to the heart, which were then surgically excised.2-4Go In the other reported case of primary tumor in the heart, the patient underwent heart transplantation.4Go This report not only demonstrates the second case of Ewing sarcoma with cardiac involvement but also the first case of resection of a primary Ewing sarcoma involving the heart without cardiac transplantation.

Various techniques of SVC reconstruction have been described. We describe a technique in which a bovine pericardial tube is fashioned with a stapling device. The pericardial tube graft is widely used in surgery for congenital heart disease for procedures such as the extracardiac Fontan and caval reconstruction, but has found limited uses in surgery for malignant thoracic diseases. This technique has been reported only twice before for SVC reconstruction for malignancy but after extended pulmonary resection.5Go This simple method facilitated our reconstruction.

This case illustrates the need for multimodality therapy for Ewing sarcoma delivered through a multidisciplinary approach involving thoracic and cardiac surgeons as well as oncologists.

References

  1. Grier HE. The Ewing family of tumors. Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 1997;44:991-1004.[Medline]
  2. Flinn RM, Foyle A, Montague TJ. Extraskeletal Ewing’s sarcoma with fatal cardiac metastasis. CMAJ 1985;133:1017-1018.[Medline]
  3. Chandramohan NK, Hussain MB, Nayak N, Kattoor J, Pandey M, Krishnankutty R. Multiple cardiac metastases from Ewing’s sarcoma. Can J Cardiol 2005;21:525-527.[Medline]
  4. Higgins JC, Katzman PJ, Yeager SB, Dickerman JD, Leavitt BJ, Tischler, MD, et al. Extraskeletal Ewing’s sarcoma of primary cardiac origin. Pediatr Cardiol 1994;15:207-208.[Medline]
  5. Spaggiari L, Veronesi G, D’Aiuto M, Tosoni A. Superior vena cava reconstruction using heterologous pericardial tube after extended resection for lung cancer. Eur J Cardiothorac Surg 2004;26:649-651.[Abstract/Free Full Text]



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