Ann Thorac Surg 1996;62:274-276
© 1996 The Society of Thoracic Surgeons
Case Report
Leiomyosarcoma of the Superior Vena Cava
Lorenzo Spaggiari, MD,
Jean-Francois Regnard, MD,
Remi Nottin, MD,
Elisabeth M. Dulmet, MD,
Michele Rusca, MD,
Paolo Bobbio, MD,
Philippe Levasseur, MD
Department of Thoracic and Vascular Surgery, Marie Lannelongue Hospital, Le Plessis Robinson, France
Accepted for publication January 26, 1996.
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Abstract
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Leiomyosarcoma of the superior vena cava is exceptional. A case in a 52-year-old man is described. A treatment by means of neoadjuvant chemotherapy, operation, and adjuvant radiotherapy was performed. This aggressive treatment has permitted the patient to obtain a relatively long survival with a good quality of life.
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Introduction
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Leiomyosarcomas of the blood vessels are rare malignant tumors. Usually considered very locally aggressive neoplasms, they metastasize generally to lungs and liver. Taking into account their radioresistance, an extensive surgical resection should be usually considered the treatment of choice. Although 50% of these malignancies are located in the inferior vena cava [1], primary leiomyosarcoma of the superior vena cava (SVC) is exceptional. We describe a case of leiomyosarcoma of the SVC in a 52-year-old man with extensive infiltration of the surrounding organs but without distant metastasis. Aggressive treatment using neoadjuvant chemotherapy, surgical resection, and adjuvant radiotherapy was performed.
In December 1993, a 52-year-old man was admitted to the hospital with a dry cough and initial signs of SVC syndrome. A computed tomographic scan revealed a diffuse mediastinal tumor involving the SVC, the left innominate vein, and almost all of the right atrium. The mediastinal part of the right upper pulmonary lobe was also involved. An esophagogram was normal. A mediastinal biopsy through a right axillary thoracotomy was performed. Sections of the biopsy specimen showed a spindle cell neoplasm growing in a diffuse fashion among fibrous strands and adipose tissue. Several large vessels outside the main mass showed a hypercellular appearance in the subendothelial region, strongly suggestive of tumor involvement. The immunohistochemical study showed the tumor was strongly positive for smooth muscle activity and vimentin and negative for S-100 protein. The combination of morphologic and immunohistochemical features was in favor of a smooth muscle tumor. The vascular component represented an indication that this tumor had arisen from smooth muscle of vessel walls. Because of the diffuse pattern of infiltration, despite its relatively low mitotic rate, the pathologist decided on a diagnosis of leiomyosarcoma of the great vessels. Considering the wide mediastinal infiltration, induction chemotherapy with doxorubicin and isophosphamide was performed. During the seven cycles of chemotherapy the patient was well.
In October 1994, the patient was restaged by means of computed tomographic scan (Fig 1
), nuclear magnetic resonance, transesophageal echocardiography, and SVC angiography (Fig 2). These investigations showed a reduction of about 40% of the tumoral mass with invasion of right atrium but without tricuspid and ventricular infiltration.
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Fig 1. . Computed tomographic scan showing the origin of the superior vena cava from the right atrium completely occluded by tumoral mass. Note the adhesion with pulmonary artery and aorta that required subadventitial dissections.
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Fig 2. . Superior vena caval angiogram. Contrast material does not appear in the right atrium. The superior vena cava is completely occluded at the origin under the azygos vein. An important collateral circle arising from the azygos vein is present.
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In November 1994, the patient was operated on. The tumor was approached through a median sternotomy. The tumoral mass infiltrated the innominate veins, occluding completely the SVC. The pericardium was partially involved as well as the superior two thirds of the right atrium. Once the pleural space was opened, we observed that the tumoral infiltration involved the internal mammary pedicle as well as the right upper lobe.
The patient was placed on cardiopulmonary bypass using an inferior vena caval cannula and an aortic cannula. He was then cooled to 29.2°C and his circulation was arrested. The right atrium was opened and widely resected until the tricuspid valve. The SVC was resected with both the innominate veins. The tumor was removed from the aorta by dissection in the subadventitial plane and from the right pulmonary artery wall. A wedge resection of the upper right lobe was performed, and the tumor was detached from the right side of the trachea. The anterolateral right atrium wall was reconstructed using an autologous pericardial patch. The right confluence between subclavian and internal jugular vein was connected to the right atrium by means of a 14-ring polytetrafluoroethylene graft and the left innominate vein was anastomosed to the graft by a 10-ring polytetrafluoroethylene graft. A polytetrafluoroethylene patch was placed on the aortic and pulmonary wall in the sites where the dissections were performed. The extracorporeal circulation was stopped 317 minutes after it began.
The early postoperative course was complicated by a hemorrhage requiring a reintervention. The patient was maintained ventilated in the intensive care unit for 21 days because of bronchial and pulmonary infection and right phrenic and laryngeal paralysis; during the same period, an arrhythmia developed that was treated by means of an epicardiac pacemaker. Thirty-two days after the operation the patient was discharged. The macroscopic and microscopic analysis of the specimens showed a leiomyosarcoma arising from the SVC.
From June 1995 until July 1995, radiotherapy (36 Gy) was performed. In September 1995 the patient was restaged, and no signs of mediastinal recurrences were found. Unfortunately, an isolated metastasis 7 cm in diameter, confirmed histologically, was observed in the right triceps. In October 1995, chemotherapy with isophosphamide was started. In January 1996, twenty-five months after the initial diagnosis and 14 months after surgical resection, the patient is still alive; his state of health is very good, and no signs of mediastinal recurrences or of metastases to lungs and liver are present. The metastasis in the triceps is stable. The chemotherapy is still in progress.
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Comment
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Only 7 cases of leiomyosarcoma of the SVC have been previously described [2–8]. The first case surgically treated was reported by Stefanini and associates [3]. The patient, a 5-year-old girl, died 3
years after the diagnosis and 1 year after operation. Davis and colleagues [4] reported the second case of resection; the patient was still alive 7 years after the original resection and 5 years after the operation for mediastinal recurrence. Although long survivals in these neoplasms are rare, the cases above reported and our present experience suggest that these tumors should be treated aggressively. In our case, chemotherapy led to an important reduction of tumoral mass. The operation heavily influenced the postoperative morbidity, and we recommend it only if a complete excision of the tumor is possible. During operation the distinction between tumoral invasion and surrounding fibrosis is often not possible; thus postoperative radiotherapy is mandatory. The biological behavior of this tumor in our case confirms its aggressive nature. Nevertheless, aggressive combined medical and surgical treatment may permit a relatively long survival with good quality of life.
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Footnotes
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Address reprint requests to Dr Regnard, Department of Thoracic and Vascular Surgery, Marie Lannelongue Hospital, 133 av de la Resistance, 92350 Les Plessis Robinson, Paris, France.
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References
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- Stefanini P, Ricci C, Benedetti-Valentini F, Faraglia V. Leiomyosarcoma of superior vena cava (a case treated successfully by surgery). Surgery in Italy 1971;1:121–8.
- 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]
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- Marini M, Tovar E, LopezFernandez MF, Pombo F, Rodriguez E. Primary leiomyosarcoma of the superior vena cava with massive thrombosis treated by local fibrinolysis. Br J Radiol 1992;65:1131–2.[Abstract/Free Full Text]
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Abstract
Introduction
