Ann Thorac Surg 1998;66:1423-1424
© 1998 The Society of Thoracic Surgeons
Case Reports
Primary malignant lymphoma of superior vena cava
Hiroaki Nomori, MDa,
Sadahiro Nara, MDa,
Shojiroh Morinaga, MDb,
Kyoko Soejima, MDc
a Department of Surgery, Saiseikai Central Hospital, Tokyo, Japan
b Department of Pathology, Saiseikai Central Hospital, Tokyo, Japan
c Department of Medicine, Tokyo Metropolitan Hiroo Hospital, Tokyo, Japan
Accepted for publication April 11, 1998.
Address reprint requests to Dr Nomori, Department of Surgery, Saiseikai Central Hospital, 1-4-17 Mita, Minato-ku, Tokyo 108-0073, Japan
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Abstract
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A 78-year-old woman suffered from superior vena cava (SVC) syndrome. Computed tomography and angiography revealed a mass within the SVC. The SVC was resected via median sternotomy, followed by reconstruction using an artificial graft. The resected specimen showed a polypoid tumor within the SVC that had invaded the wall of the SVC. Histologic diagnosis was diffuse large-cell non-Hodgkin’s lymphoma. Most surrounding lymph nodes showed reactive swelling, but one showed a partial microscopic metastasis. These pathologic findings indicated that the tumor was an SVC-originating malignant lymphoma. The patient is now alive and tumor-free 65 months after the operation.
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Introduction
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Superior vena cava (SVC) syndrome due to malignant lymphoma is usually caused by SVC obstruction after a tumor from a primary anterior mediastinal lesion has spread. We present a case of SVC-originating malignant lymphoma treated by operation followed by chemotherapy.
A 78-year-old woman complained of facial and bilateral arm swelling in March 1992, and was admitted to Saiseikai Central Hospital on April 28, 1992. Chest computed tomography showed the SVC to have expanded from a central to a peripheral site with a low-density intraluminal mass, and paratracheal lymph nodes to be swollen (Fig 1). A contrast superior vena cavogram showed an obstructed SVC and numerous collateral vessels (Fig 2). Because of immediate worsening of the patient’s condition, an expandable metallic stent was introduced to treat SVC obstruction. Transvenous biopsy of the tumor revealed malignancy, but no further diagnosis could be made because of the occurrence of compressive artifacts. Systemic examination revealed no other tumors.
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Fig 1. Chest computed tomogram showing an expanded superior vena cava with an intraluminal mass (arrow) and enlarged paratracheal lymph node.
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An operation was conducted on May 7, 1992, via median sternotomy with right fourth intercostal thoracotomy. The SVC was enlarged to 4 cm in diameter, but no invasion of adjacent organs was seen. The bilateral brachiocephalic veins, azygos vein, and SVC including part of the right atrium were resected, and reconstruction was implemented with a ringed, expanded polytetrafluoroethylene graft 14 mm in diameter and 10 cm long between the right brachiocephalic vein and the right atrium.
The specimen showed a polypoid tumor within the SVC that projected into the right atrium, azygos vein, and right brachiocephalic vein. The tumor had invaded a wall of the SVC, which was considered to be the site of tumor origin (Fig 3). The surgical margin at the azygos vein was positive for malignancy, but no invasion of adjacent organs was seen. The pathologic diagnosis was diffuse large-cell non-Hodgkin’s lymphoma with sclerosis. The immunohistologic staining showed the lymphoma cells to be B cells. Most paratracheal lymph nodes showed reactive swelling, and one showed a partial microscopic metastasis. From these findings, we diagnosed the tumor as SVC-originating malignant lymphoma.
The postoperative course was satisfactory, requiring no anticoagulant therapy, and an SVC angiogram 29 days after the operation showed good patency. Two courses of postoperative chemotherapy using cyclophosphamide, doxorubicin, vincristine, and prednisolone were administered. Forty-one months after the operation, the patient suffered from scleroderma limited to both hands. Follow-up computed tomography 55 months after the operation revealed good graft patency, but showed tumor recurrence at the mediastinum. The patient was treated with six additional courses of chemotherapy at Tokyo Metropolitan Hospital. She is now living tumor-free 65 months after the operation, and the symptoms of scleroderma have stabilized since their onset.
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Comment
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Although primary cardiac malignant lymphoma has been reported [1–3], malignant lymphoma rarely originates in the great vessels. Here we present a case of SVC-originating malignant lymphoma.
Patients with scleroderma have been reported to show an increased incidence of lung, breast, hematopoietic, and lymphoproliferative malignancies [4]. Although several reports have been made of malignant lymphoma associated with scleroderma [5], most patients suffer first from scleroderma and then have development of lymphoma within 3 years. Our patient, however, suffered from malignant SVC lymphoma, then had development of scleroderma 41 months after the operation.
We do not usually use anticoagulant therapy after artificial graft replacement for the great veins, including the SVC, internal carotid, and subclavian veins, for the following reasons: (1) the blood flow in those great veins is sufficient to prevent thrombokinesis and (2) a large-diameter ringed graft is usually used for straight-line vessel replacement. This has met with success in 15 patients.
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References
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- Zaharia L., Gell P.S. Primary cardiac lymphoma. Am J Clin Oncol 1991;14:142-145.[Medline]
- Roller M.B., Manoharan A., Lvoff R. Primary cardiac lymphoma. Acta Haematol 1991;85:47-48.[Medline]
- Constantino A., West T.E., Gupta M., Loghmanee F. Primary cardiac lymphoma in a patient with acquired immune deficiency syndrome. Cancer 1987;60:2801-2805.[Medline]
- Duncan S.C., Winkelman R.K. Cancer and scleroderma. Arch Dermatol 1979;115:950-955.[Abstract/Free Full Text]
- Hall S.W., Gillespie J.J., Tencznski T.F. Generalized lipodystrophy, scleroderma, and Hodgkin’s disease. Arch Intern Med 1978;138:1303-1304.[Abstract/Free Full Text]
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Abstract
Introduction
