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Ann Thorac Surg 1996;61:1840-1841
© 1996 The Society of Thoracic Surgeons

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Case Report

Leiomyosarcoma of the Pulmonary Veins With Extension to the Left Atrium

Departments of Surgery, Pathology, and Internal Medicine, Faculty of Medicine, University of Concepción, Concepción, Chile

Accepted for publication December 5, 1995.

	Abstract

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A leiomyosarcoma of the right pulmonary vein in a 43-year-old woman extended to the right atrial wall and compromised the posterior mitral leaflet. Successful surgical treatment was accomplished with a right pneumonectomy, partial resection of the left atrial wall, and mitral valve replacement under cardiopulmonary bypass. Six months later a mediastinal recurrence with extension to the left hemithorax was treated with resection and postoperative radiotherapy.

	Introduction

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Soft tissue sarcoma is the most common malignant neoplasm of the heart, pericardium, pulmonary, and great vessels [1]. Primary tumors of the pulmonary arteries and veins are rare and seldom diagnosed during the patient's lifetime [2–4]. Ninety-three cases of primary sarcomas of the pulmonary artery were reported in 1990 [3], yet only two pulmonary vein tumors-an alveolar soft tissue sarcoma [4] and a pulmonary vein myxoid leiomyosarcoma [5]-were reported. In this article we report successful treatment of a leiomyosarcoma of the right pulmonary vein that extended into the left atrium and posterior mitral leaflet.

A 43-year-old woman with a month's history of cough, dyspnea, and weight loss followed by hemoptysis was referred for evaluation. Due to hemoptysis chest films showed bilateral infiltrates. Bronchoscopy revealed bleeding from the right superior lobe. The severity of hemoptysis required blood transfusion. Lung scintigraphy demonstrated severe right hypoperfusion. Pulmonary arteriography showed slowing of the venous drainage from the right lung. A hemodynamic study gave a pulmonary capillary wedge pressure of 41 mm Hg and a pulmonary artery pressure of 86/38 mm Hg (mean, 56 mm Hg). Cardiac output was 6.4 L/min and the cardiac index, 4.3 L • min^-1 • m^-2.

The computed tomographic scan and the echocardiogram demonstrated a tumor in the left atrium. The nuclear magnetic resonance study showed a tumor originating from the right pulmonary hilum, extending into the pulmonary veins, and finally filling the left atrial chamber (Fig 1).

View larger version (154K): [in this window] [in a new window]   Fig 1. . Magnetic nuclear resonance study showing tumor arising in the right pulmonary veins and filling the left atrium. (AO = aorta; AP = pulmonary artery; VI = left ventricle.)

Postoperatively cardiac function and ventilation were adequate and initially the patient had a satisfactory recovery. A week later she was reexplored for mediastinitis, and an extended right pleurostomy was performed to control a right-sided empyema. The patient was discharged 2 months later.

A computed tomographic scan done 3 months after operation did not show any residual tumor; however, a second scan performed 6 months later showed a mediastinal mass extending into the left hemithorax. A biopsy was performed through a small left thoracotomy, and tissue specimen obtained possessed identical histologic properties as the tumor resected 6 months earlier.

On the basis of a normal cerebral scan we performed a left thoracotomy, resecting a mediastinal tumor that showed no infiltration of the surrounding structures. Postoperative radiotherapy was done. One year later the patient is alive.

The tissue resected from the left atrium was 5.2 x 4.2 x 3.3 cm in diameter, white, firm, and nodular. The tumor was partially covered by an endothelial layer. Areas of myxoid appearance were seen. No necrosis was present. The right lung (120 g in weight and 20 x 6 x 12.7 cm in dimensions) showed intraluminal occlusive white tissue filling the right superior and inferior pulmonary veins. The tumor was firmly attached to the walls of the veins but no invasion into the pulmonary parenchyma was seen.

The histologic appearance of the tumor from the left atrium and right pulmonary veins was similar. There was a high cellular proliferation of spindle cells forming ill-defined bundles. The nuclei were atypical with a cigar-shaped appearance. Mitotic figures were frequently seen. The tumor cells invaded the muscular layer of the wall of the veins. No microscopic invasion outside of the vessel was identified. With immunoperoxidase technique there was a positive reaction to vimentin and muscle-specific actin (HHF35) in the cytoplasm of the tumor cells. By transmission electron microscopy dense filaments typical of the smooth muscle differentiation were found in the cytoplasm of the neoplastic cells. No metastases were found in the lymph nodes.

The tumor resected at recurrence was 12.5 x 8 x 4 cm in dimensions, white, myxoid, firm, and well-circumscribed. It was histologically similar to the pulmonary veins tumor removed 6 months earlier. The repeated immunostaining and electron microscopic studies also showed identical patterns.

	Comment

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The topography of the tumor could be clearly identified as originating from the right inferior pulmonary vein, where the involvement was obvious. The tumor was a highly cellular neoplasm of spindle cells with blunt ending atypical nuclei-forming irregular bundles, which is considered a classic histologic appearance of smooth muscle. Also, the smooth muscle differentiation is supported by the positive reaction to muscle-specific actin found in the cytoplasm of the tumoral cells and by the presence of the typical filaments seen under the electron microscope.

The decision to resect the neoplasm was based on the fact that resection of vascular sarcomas, when possible, is technically challenging, but may result in cure or palliation [1]. Sarcomas originating in the pulmonary arteries have poor prognosis due to local recurrences with survival seldom being longer than 18 months [2, 3]. In this case the recurrence occurred in a mediastinal area not anatomically related to the primary tumor. Therefore, the only possible explanation for the local recurrence was due to tumor cell implantation during sternotomy.

Initially, we did not feel the need to use chemotherapy or radiotherapy because we thought there was not enough evidence of their efficacy in this setting [3], but after the recurrence we changed our minds, because it has been published that chemotherapy and radiation therapy can relieve symptoms as well as prolong survival [1].

	Footnotes

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Address reprint requests to Dr Gyhra, Department of Surgery, Faculty of Medicine, Universidad de Concepción, Casilla 60-C Concepción, Chile.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol 1994;10:374–82.[Medline]
2. Ramp U, Gerharz CD, Iversen S, Scweden F, Steppling H, Gabbert HE. Sarcoma of the pulmonary artery: report of two cases and a review of the literature. J Cancer Res Clin Oncol 1992;118:551–6.[Medline]
3. Kruger I, Borrowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990;38:91–5.[Medline]
4. Tsutsumi Y, Deng YL. Alveolar soft part sarcoma of the pulmonary vein. Acta Pathol Jpn 1991;41:771–7.[Medline]
5. Gonzalez-Campora R, Rubi-Uria J, Mora-Marin J, Hevia A, Galera-Davidson H. Pulmonary vein myxoid leiomyosarcoma. Pathol Res Pract 1989;185:900–6.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Alberto S. Gyhra Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Gyhra, A. S. Articles by Carrillo, H. Search for Related Content PubMed PubMed Citation Articles by Gyhra, A. S. Articles by Carrillo, H.