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Ann Thorac Surg 2009;87:1616-1618. doi:10.1016/j.athoracsur.2008.09.052
© 2009 The Society of Thoracic Surgeons
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Case Reports

Intracardiac Invasive Thymoma Presenting as Superior Vena Cava Syndrome

Ahmad Ali Amirghofran, MDa, Abbas Emaminia, MDa,*, Shahrzad Rayatpisheh, MDa, Seyed Ali Malek-Hosseini, MDb, Yahya Attaran, MDc

a Division of Cardiovascular Surgery, Shiraz University of Medical Sciences, Shiraz, Iran
b Department of General Surgery, Shiraz University of Medical Sciences, Shiraz, Iran
c Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran

Accepted for publication September 11, 2008.

* Address correspondence to Dr Emaminia, Division of Cardiovascular Surgery, Department of Surgery, Faghihi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran (Email: emaminia{at}gmail.com).


   Abstract
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A case of invasive thymoma with intra-caval and intracardiac extension presenting as superior vena cava syndrome is reported. The tumor is excised on cardiopulmonary bypass, and superior vena cava is bypassed using a Dacron graft (DuPont, Wilmington, DE). Five-year follow-up of the patient showed a patent graft.


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Superior vena cava (SVC) syndrome is typically associated with advanced malignancies, and it is very infrequently associated with thymomas [1]. Invasive thymomas are characterized by infiltrative growth and extension into the surrounding structures [2]. We describe a rare case of invasive thymoma with intra-caval and intracardiac extension into the right atrium.

The patient was a 53-year-old man who was admitted to the hospital for work-up of his face and upper extremity edema. Chest roentgenogram showed mediastinal widening on the right margin. Computed tomographic scan revealed a mass in the anterior mediastinum, filling the left brachiocephalic vein and extending to the right atrium through the SVC (Fig 1). Due to its extensive nature and the potential risks associated with the location of the mass, a decision was made to take the patient to the operating room emergently without any preoperative chemotherapy or radiation therapy.


Figure 1
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  		Fig 1. Computed tomographic scan showing a large, well-defined round, soft-tissue density in the superior vena cava right atrium junction (arrow).

 
The procedure was performed through a mid-sternotomy approach. A firm, large white mass was discovered in the anterior mediastinum with extension into the middle and posterior mediastinum invading the left brachiocephalic vein, the SVC, the right hilum of the lung, the right phrenic nerve, and the pericardium. The first step was to resect the accessible bulk of the tumor over the aorta, the SVC and the left brachiocephalic vein. Cardiopulmonary bypass was instituted after cannulation of the right femoral vein and the ascending aorta. The upper body venous drainage was established through a 10-mm Dacron graft (DuPont, Wilmington, DE) and anastomosed to the left brachiocephalic vein. The right atrium was then opened. A large mass (measuring 10 x 10 cm) was seen in the atrium with its tail in the right ventricle, partially obstructing the tricuspid valve. The intracardiac portion of the mass was resected through the mid-SVC. The mass had not invaded any cardiac structures. The SVC was then bypassed by anastomosing a Dacron graft to the right atrial appendage, which effectively reduced the central venous pressure. The patient was uneventfully weaned off cardiopulmonary bypass.

Pathologic examination revealed a type AB thymoma (World Health Organization classification), which had directly invaded the left brachiocephalic vein. The patient was discharged home on postoperative day 4, and further management through chemotherapy and radiation therapy was pursued.

In the most recent computed tomographic scan 5 years after the operation, the diffuse and distant metastasis of the original tumor was remarkable. The Dacron graft (DuPont) was patent and the patient was free from any signs and symptoms indicative of SVC syndrome.


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Invasive thymomas usually infiltrate the adjacent organs in the mediastinal pleura, including the lungs, pericardium, great vessels, and the heart. However, extrathoracic metastases are very uncommon, occurring in only approximately 5% of cases [3].

We believe that there have been 15 other cases of intracardiac thymomas reported in the literature aside from this report. The longest reported follow-up was 36 months, in which the patient returned with recurrence of SVC syndrome. The patient in this report was followed-up for 5 years, and the latest computed tomographic scan showed the graft to be patent.

The goal of surgery in such cases is to restore the blood flow from the brachiocephalic veins to the right atrium. This can be performed either by cleaning the SVC from the tumor bulk or through extra-anatomical grafts. Both biologic and synthetic materials, such as autologous vein, autologous, or bovine pericardium, and expanded polytetrafluoroethylene have been used in the venous system [4–6]. Doty and colleagues [4] believe in superiority of autologous vein, but more time is required to reconstruct a spiral vein graft and a long suture line, which may increase the thrombogenicity of the graft. On the other hand, expanded polytetrafluoroethylene grafts used in these patients, have reportedly proven to have excellent short-term and long-term results [5, 6]. However, the problem of flexion and kinking are prominent with long conduits [7].

Dong Lu and colleagues [7] described a case of SVC reconstruction using valved bovine jugular vein with excellent 42-month patency, although other reports discourage the use of such conduits due to thrombosis [8].

In conclusion, we report a rare case with invasive thymoma to the SVC and right atrium that underwent en bloc excision of the tumor with SVC being bypassed using a Dacron graft with excellent 5-year patency. Use of extra-anatomical conduits in the venous system is most probably safe and at times may even be lifesaving.


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  1. Chen JC, Bongard F, Klein SR. A comtemporary perspectine on superior vena cava syndrome Am J Surg 1990;160:207-211.[Medline]
  2. Cohen DJ, Ronnigen LD, Graeber GM, et al. Management of patients with malignant thymoma J Thorac Cardiovasc Surg 1984;87:301-307.[Abstract]
  3. Airan B, Sharma R, Iyer KS, et al. Malignant thymoma presenting as intracardiac tumor and superior vena caval obstruction Ann Thorac Surg 1990;50:989-991.[Abstract/Free Full Text]
  4. Doty JR, Flores JH, Doty DB. Superior vena cava obstruction: bypass using spiral vein graft Ann Thorac Surg 1999;67:1111-1116.[Abstract/Free Full Text]
  5. Magnan PE, Thomas P, Giudicelli R, Fuentes P, Branchereau A. Surgical reconstruction of the superior vena cava Cardiovasc Surg 1994;2:598-604.[Medline]
  6. Spaggiari L, Thomas P, Magdeleinat P, et al. Superior vena cava resection with prosthetic replacement for non-small cell lung cancer: long-term results of a multicentric study Eur J Cardiothorac Surg 2002;21:1080-1086.[Abstract/Free Full Text]
  7. Dong Lu W, Yu FL, Wu ZS. Superior vena cava reconstruction using bovine jugular vein conduit Eur J Cardiothorac Surg 2007;32:816-817.[Abstract/Free Full Text]
  8. Schoof PH, Koch AD, Hazekamp MG, Waterbolk TW, Ebels T, Dion RA. Bovine jugular vein thrombosis in the Fontan circulation J Thorac Cardiovasc Surg 2002;124:1038-1040.[Free Full Text]



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