Ann Thorac Surg 2000;70:1713-1714
© 2000 The Society of Thoracic Surgeons
Case report
Long-term survival after radical resection of a primary angiosarcoma of the innominate vein
Ralph I. Rückert, MDa,
Thomas J. Kröncke, MDb,
Klaus Bürger, MDc
a Department of Surgery, Humboldt University Medical School, Campus Charité Mitte, Berlin, Germany
b Department of Radiology, Humboldt University Medical School, Campus Charité Mitte, Berlin, Germany
c Private Practice, Berlin, Germany
Address reprint requests to Dr Rückert, Department of Surgery, Humboldt University Medical School, Campus Charité Mitte, Schumannstr 20/21, D-10117 Berlin, Germany
e-mail: ralph-i.rueckert{at}charite.de
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Abstract
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The case updated here demonstrates the longest survival that has been observed so far after radical resection of primary angiosarcoma of the left innominate vein, which is the second case that has ever been reported to date. Radical resection was performed after preoperative radiotherapy. The venous continuity was restored by expanded polytetrafluoroethylene graft interposition. This case underscores that the prognosis of primary venous sarcoma, though fatal in the majority of patients, may be better in individual cases when radical surgery with curative intention is achieved.
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Introduction
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Primary angiosarcoma of the great veins is exceedingly rare [1]. Apart from the inferior vena cava as the most common venous site of origin, other primary venous sites such as the axillary vein [2] or the superior vena cava [3] have been reported only sporadically. The case updated here demonstrates long-term survival after radical resection of primary angiosarcoma of the left innominate vein, which is the second case that has ever been reported to date.
The details of the case have been reported previously [4]. Briefly, a then 39-year-old woman was transferred from another hospital to the Department of Surgery of Humboldt University Medical School (Charité) in November 1989 for further therapeutic management because of a malignant hemangioendothelioma in the anterior mediastinum. An earlier computed tomography (CT) performed in 1986 had yielded the diagnosis of "enlargement of the thymic gland" without further consequence. Only 2.5 years later, the patient again consulted a doctor because of dyspnea. History and clinical examination revealed the symptoms of superior vena caval syndrome with massive inflow obstruction. After CT-guided puncture of the tumor in the anterior mediastinum (Fig 1) had revealed malignant cells, radiotherapy with 30 Gy was performed without tumor regression. In September 1989, a diagnostic thoracotomy was performed in another hospital, from where the patient was transferred to our department after the diagnosis of angiosarcoma had been established.
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Fig 1. Bolus-enhanced axial CT scan of the chest demonstrating the tumor in the anterior mediastinum caused by an angiosarcoma of the left innominate vein (arrowheads).
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After median sternotomy, the tumor of the anterior mediastinum was intraoperatively identified as originating from the left innominate vein. The tumor included the entire left innominate vein from the junction of the subclavian and internal jugular vein to the right atrium. The right innominate as well as internal jugular vein were completely thrombosed. The tumor had caused a paresis of the right phrenic nerve. Complete resection was achieved and the venous inflow restored by interposition of a 12-mm prosthetic expanded polytetrafluoroethylene (ePTFE) graft (W.L. Gore & Associates, Flagstaff, AZ) between the venous confluence of the left internal jugular and subclavian vein and the right atrium. The definite histomorphological diagnosis of the resected specimen revealed a sarcoma of endothelial origin. After an uneventful postoperative course, the patient was followed with clinical examinations and CT scans at regular 6-month intervals. Ten years after the operation, the patient is doing well, and CT as well as magnetic resonance angiography (Fig 2) continue to document no recurrent or metastatic tumor growth and patency of the ePTFE graft.
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Fig 2. Magnetic resonance angiography 10 years after radical resection of the primary angiosarcoma with restoration of flow by ePTFE interposition. The Gadolinium-DTPA-enhanced targeted maximal-intention projection demonstrates the patent ePTFE graft (arrowheads) without recurrent tumor.
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Comment
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Primary sarcomas of the great vessels are rare, with the inferior vena cava, however, representing the second most common site [1]. Nevertheless, malignant primary vein tumors of other origins are extremely rare. Primary angiosarcoma of the innominate vein was first described in 1985 [5], and to date, only two further cases have been reported, including the case updated here [4, 6]. Though malignant neoplasms involving the great veins have a variety of histologic diagnoses, leiomyosarcoma is by far the most common one. However, all three cases of malignant innominate vein tumors showed the pattern of an endothelial neoplasm (angiosarcoma or malignant hemangioendothelioma).
The first case, presented by Miller and associates [5], demonstrated long-term survival of at least 8 years, which is confirmed by the case reported here, with the longest survival that has been observed so far in a patient after radical surgery for primary angiosarcoma of the innominate vein. The possible contribution of preoperative radiotherapy to successful long-term survival cannot be assessed exactly but has to be considered in the case reported here. This case underscores that the prognosis of primary venous sarcoma, though fatal in the majority of patients, may be better in individual cases when radical surgery with curative intention is achieved.
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References
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Burke A., Virmani R. Sarcomas of the great vessels. Cancer 1993;71:1761-1773.[Medline]
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Alosco T., Sinning H., Harwick R., Locke J.L., Tang C.K. Angiosarcoma of the axillary vein. Cancer 1989;64:1301-1303.[Medline]
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Abratt R.P., Williams M., Raff M., Dodd N.F., Uys C.J. Angiosarcoma of the superior vena cava. Cancer 1983;52:740-743.[Medline]
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Bürger K., Scholz H., Luther B. Angiosarkom der V. brachiocephalica. Angio 1990;12:143-145.
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Miller M.M., Munnell E.R., Poston A., Harkey M.R., Grantham R.N. Primary angiosarcoma of the innominate vein. J Thorac Cardiovasc Surg 1985;90:148-150.[Abstract]
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Rytina E.R., Govil Y.K., Sabanathan K., Ball R.Y. Intimal sarcoma of the right brachiocephalic vein presenting as the superior vena caval syndrome. J Clin Pathol 1996;49:347-349.[Abstract/Free Full Text]
Accepted for publication May 13, 2000.
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Abstract
Introduction
