Ann Thorac Surg 1995;60:1415-1417
© 1995 The Society of Thoracic Surgeons
Case Reports
Leiomyosarcoma of the Superior Vena Cava and Azygos Vein
James M. Levett, MD,
William G. Meffert, MD,
Wilson W. Strong, MD,
A. Curtis Hass, MD,
Ruth A. Macke, MD,
Gordon G. Berg, MD,
Mary Anne Nelson, MD,
Chirantan Ghosh, MD
St. Luke's Methodist Hospital, Cedar Rapids, Iowa
Accepted for publication May 12, 1995.
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Abstract
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A 40-year-old woman presented with facial swelling and pressure sensation of the ears and sinuses. Chest roentgenography revealed a right paratracheal mass, which was confirmed by a venogram, and transjugular biopsy showed low-grade leiomyosarcoma. The superior vena cava was resected and reconstructed using a spiral vein graft. Pathologic evaluation revealed a low-grade leiomyosarcoma arising from both the superior vena cava and the azygos vein with clear margins.
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Introduction
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Leiomyosarcomas are relatively rare malignant tumors of blood vessels, with the majority arising from veins. The most commonly involved vein has been the inferior vena cava, although previous reports have documented leiomyosarcomas arising from other veins, including the common iliac, ovarian, greater saphenous, antecubital, external iliac, and superior vena cava (SVC) [1–4]. Leiomyosarcoma of the SVC is rare, with few cases previously reported [1–4].
A 40-year-old woman was admitted with a complaint of facial swelling and pressure sensation of the ears and sinuses. A chest roentgenogram showed a right paratracheal mass, and a computed tomographic scan of the chest revealed a filling defect in the SVC (Figs 1, 2
) without other significant mediastinal abnormalities. A venogram subsequently documented a 3 cm x 5 cm filling defect in the SVC with involvement of the azygos vein (Fig 3). Percutaneous transvenous biopsy revealed this mass to be a spindle cell tumor. Computed tomographic scan of the head and liver function tests were within normal limits, and the patient was referred for surgical therapy.
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Fig 1. . Preoperative chest roentgenogram demonstrating a large right paratracheal mass and mediastinal enlargement consistent with tumor in the superior vena cava and enlargement of the azygos vein secondary to superior vena caval obstruction.
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Fig 2. . Preoperative computed tomographic scan of the chest with contrast showing obstruction of the superior vena cava secondary to intraluminal mass.
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Fig 3. . Preoperative superior vena cavogram demonstrating subtotal occlusion of the superior vena cava secondary to intraluminal mass.
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A median sternotomy was performed, and after inspection of the tumor mass we elected to replace the entire SVC. Measurements of the SVC were taken, and the greater saphenous vein was harvested from the medial aspect of the left thigh. A spiral vein graft was constructed over an appropriately sized chest tube and sewn together using running 7-0 polypropylene, and 9,000 units of heparin was given. The SVC was then resected and found to have a tumor mass occluding nearly the entire extent of the SVC from the innominate artery to the right atrium. It was not particularly adherent to the sides of the SVC, but the extension into the azygos vein was not easy to dissect, and this gave the clinical impression that the mass originated in the azygos vein.
The azygos vein was dissected approximately 15 cm until a normal margin was found on frozen section. The SVC was reconstructed using the spiral vein graft with running 5-0 polypropylene. A shunt was not used, and central venous pressure was monitored and maintained at approximately 25 to 28 mm Hg during the clamping period.
The resected specimen was evaluated by the Department of Pathology at St. Luke's Hospital and the University of Iowa Hospitals and Clinics. The final pathologic diagnosis was low-grade leiomyosarcoma with margins of both the SVC and the azygos vein free of tumor. The SVC specimen was 5.2 cm x 2.8 cm x 2.6 cm, and in both the SVC and the azygos vein, the tumor was identified as arising from the wall in several areas. In one area, the tumor appeared to infiltrate outside of the azygos vein and into fat. A lymph node removed from the area behind the SVC was negative for malignancy. Because the tumor was apparently arising from the wall in areas of both the SVC and the azygos vein, it could not be determined whether the malignancy originally arose from the azygos vein or the SVC.
Postoperatively, the patient was given low-dose aspirin and warfarin. Several days after the operation, she had a pressure sensation in her ears, and a follow-up venogram was performed that showed patency of the spiral vein graft replacing the SVC (Fig 4). A moderate right pleural effusion noted on the fourth postoperative day was treated with a thoracentesis, and the patient was discharged on the fifth postoperative day. Discharge medications included aspirin 80 mg per day orally, prednisone on a tapering dosage of 5 mg per day orally, and warfarin as directed.
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Fig 4. . Postoperative superior vena cavogram showing the widely patent saphenous vein superior vena caval graft.
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The patient was referred for radiation therapy with a diagnosis of low-grade leiomyosarcoma, stage T2, N0 M0. The patient received a dose of 6,000 cGy in a series of 30 daily treatments. She tolerated the treatment very well until the last few days of the treatment, when esophagitis developed, which was treated medically. At the present time, she is free of disease and doing well.
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Comment
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Leiomyosarcoma is a rare malignancy of blood vessels characterized as a smooth muscle tumor arising from the media. According to a study by Dzsinich and associates [5], only 197 patients with primary venous leiomyosarcoma had been reported by 1992. Leiomyosarcomas involving vessels account for about 2% of all leiomyosarcomas and involve large veins approximately five times more than arteries [6]. Numerous veins have been involved, with the majority being in the inferior vena cava in women [7]. The pulmonary artery has been reported in more than 10 cases, and systemic arteries have included the aorta, femoral, splenic, and iliac [6]. According to Kevorkian and Cento [6], the order of involvement in decreasing frequency is inferior vena cava and other large veins, pulmonary artery, and large systemic arteries. A report by Stout [8] in 1961 described involvement of the azygos vein, but we are unable to find other studies implicating the azygos vein as a site of origin of a leiomyosarcoma.
The present report is unusual because of the presentation of SVC syndrome secondary to a tumor arising from both the azygos vein and the SVC. Superior vena cava replacement using a spiral vein graft was reported by Anderson and Li in 1983 [4], and at that time a leiomyosarcoma arising from the SVC was resected and replaced with a saphenous spiral vein graft using a heparin-bonded shunt. In the present case, the SVC replacement was accomplished without the use of a shunt, which simplified the procedure and resulted in an uneventful recovery. Several other case reports [3, 4, 9] have documented leiomyosarcoma of the SVC, with 1 case by Davis and associates [3] including resection of the tumor and SVC repair using a saphenous vein patch graft.
We used the standard formula suggested by Doty and associates [10] for determining the length of the spiral vein graft by multiplying the ratio of SVC diameter to average saphenous vein graft diameter by the length of SVC to be replaced. It was our clinical impression at the time of the operation that this formula resulted in appropriate length, but the overall diameter was larger than was needed by a factor of about 20%. We therefore recommend selecting a stent slightly smaller in diameter than the SVC being replaced because the spiral vein graft has a tendency to stretch when subjected to normal venous pressure.
In conclusion, this report describes successful replacement of the SVC using the spiral vein graft technique for leiomyosarcoma arising in the SVC and azygos vein causing subtotal obstruction of the SVC.
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Footnotes
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Address reprint requests to Dr Levett, Department of Surgery, Lutheran General Hospital, 1775 W Dempster St, Park Ridge, IL 60068.
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References
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- Szasz IJ, Barr R, Scobie TK. Leiomyosarcoma arising from veins: two cases and a review of the literature on venous neoplasms. Can J Surg 1969;12:415–9.[Medline]
- Mingoli A, Feldhaus RJ, Cavallaro A, Stipa S. Leiomyosarcoma of the inferior vena cava: analysis and search of world literature on 141 patients and report of three new cases. J Vasc Surg 1991;14:688–99.[Medline]
- Davis GL, Bergmann M, O'Kane H. Leiomyosarcoma of the superior vena cava. A first case with resection. J Thorac Cardiovasc Surg 1976;72:408–12.[Abstract]
- Anderson RP, Li W. Segmental replacement of superior vena cava with spiral vein graft. Ann Thorac Surg 1983;36:85–8.[Abstract]
- Dzsinich C, Gloviczki P, van Heerden JA, et al. Primary venous leiomyosarcoma: a rare but lethal disease. J Vasc Surg 1992;15:595–603.[Medline]
- Kevorkian J, Cento DP. Leiomyosarcoma of large arteries and veins. Surgery 1973;73:390–400.[Medline]
- Burke AP, Virmani R. Sarcomas of the great vessels. Cancer 1993;71:1761–73.[Medline]
- Stout AP. Sarcomas of the soft tissues. CA 1961;11:210–31.[Medline]
- Lupetin AR, Dash N, Beckman I. Leiomyosarcoma of the superior vena cava: diagnosis by cardiac gated MR. Cardiovasc Intervent Radiol 1986;9:103–5.[Medline]
- Doty DB, Doty JR, Jones KW. Bypass of superior vena cava. Fifteen years' experience with spiral vein graft for obstruction of superior vena cava caused by benign disease. J Thorac Cardiovasc Surg 1990;99:889–96.[Abstract]
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Ann. Thorac. Surg.,
October 1, 1998;
66(4):
1405 - 1405.
[Abstract]
[Full Text]
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Abstract
Introduction
