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Ann Thorac Surg 1995;59:1487-1490
© 1995 The Society of Thoracic Surgeons

Primary Pulmonary Artery Sarcoma: A Report of Six Cases

Division of Cardiothoracic Surgery, University of California at San Diego Medical Center, San Diego, California

Accepted for publication February 16, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comments References

 
Pulmonary artery tumors are rare and a frequently overlooked cause of pulmonary artery occlusion. The presentation is one of progressive pulmonary dysfunction and right ventricular failure. The diagnosis seldom is made preoperatively. We report 6 cases of primary sarcoma of the pulmonary artery identified at operation, which were treated surgically. Resection with or without adjuvant therapy currently offers the only chance for survival. Emphasis must be placed on earlier identification of these tumors.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comments References

 
See also page 1490.

Primary tumors of the pulmonary arteries are rare. The clinical presentation closely resembles several more common conditions, so they seldom are diagnosed preoperatively. The etiology of these tumors remains obscure. Newer diagnostic techniques have helped in establishing earlier identification. The histopathologic characteristics are variable and precise identification is often difficult. Treatment is primarily surgical with the role of adjuvant therapy as yet not clearly defined. The prognosis remains poor.

Our institution has been engaged in a program for surgical correction of chronic pulmonary thromboembolic disease since 1970, and recently has reported the experience and results with 150 pulmonary thromboendarterectomy operations [1]. Patients are referred from all areas for evaluation of their progressive pulmonary dysfunction associated with pulmonary hypertension. In this pursuit we have encountered 6 cases of primary sarcoma of the pulmonary artery within the last 6 years. We present these cases, as well as their diagnosis and management, and review the literature.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comments References

 
This series includes 4 men and 2 women ranging from 37 to 73 years of age who were identified over a 6-year period. All patients presented with progressive dyspnea; 1 with associated cough, another with associated cough and pleuritic pain, and 1 with associated chest pain and pleural effusion. The average duration of symptoms was 17 months. All patients underwent evaluation for chronic thromboembolic disease, with only 2 patients having strong evidence suggesting previous pulmonary embolism. Each patient had a preoperative chest radiologic examination, with abnormalities being identified in 2 patients, which consisted of scattered pulmonary nodules. All patients had abnormalities identified on ventilation-perfusion scintiscan, as well as on pulmonary angiography (Fig 1). A single patient had aspiration biopsies during pulmonary angioscopy, which were suggestive but not diagnostic of malignancy. At operation in all 6 cases there was evidence of bilateral disease. Operative procedures consisted of excision of all gross tumor (endarterectomy of vessel) in 4 patients, limited tumor resection (partial endarterectomy leaving residual tumor) in the fifth, and right ventricular outflow reconstruction and distal bypass into the right pulmonary artery with a valved aortic homograft (secondary to unresectable locally invasive disease) in the remaining patient. A single patient in whom it was thought the tumor had been resected underwent left pneumonectomy for isolated pulmonary metastases at an outside institution 2 months after the initial operation.

View larger version (169K): [in this window] [in a new window]   Fig 1. . Pulmonary angiogram showing complete occlusion of left main pulmonary artery. Tumor also was found in the right pulmonary artery at operation.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comments References

	Comments

Top Footnotes Abstract Introduction Material and Methods Results Comments References

The first case of pulmonary artery sarcoma was reported by Mandelstamm in 1923, and since then, there have been few reports in the literature [3–5], with the total number of cases reported only approaching 100. Although rare, the true incidence is unknown and probably understated. In our experience of pulmonary hypertension due to thromboembolic disease, patients generally do not present until well over half the pulmonary vasculature has been occluded, and even in the late stages of the disease the diagnosis is often missed. It is therefore likely that there are patients in whom pulmonary hypertension due to tumor is undiagnosed. Our interest in the surgical treatment of chronic thromboembolic disease has led to our encountering 6 of these patients in a 6-year period.

The clinical picture in our patients varied from mild dyspnea on exertion to respiratory distress and florid right heart failure. The usual history of patients with pulmonary artery tumors is one of progressive dyspnea, often with associated cough and chest pain. There may be coexisting signs of increasing right ventricular dysfunction. The presence of flow bruits over the pulmonary arteries is also suggestive. This clinical history is strikingly similar to that of patients with chronic thromboembolic disease. Although there was no strong history of previous deep venous thrombosis or pulmonary embolism in 4 of our 6 patients, only 50% of our pulmonary thromboendarterectomy patients here have no prior history of thromboembolism or deep venous thrombosis [1].

Once the diagnosis is entertained, an extensive work-up may be required. The evaluation of our patients included chest radiologic examination, ventilation-perfusion scintiscan, echocardiogram, right heart catheterization, bilateral pulmonary angiography, and often pulmonary angioscopy. Chest radiologic examination is usually nonspecific. However, hilar pulmonary artery dilatation, increased heart size, and a changed pulmonary vascular pattern, as well as secondary lesions, may arouse suspicion of a pulmonary artery tumor. Atypical patterns for pulmonary embolism seen on ventilation-perfusion scans, as well as pedunculated or lobulated lesions with ``to and fro'' motion, or smooth tapering and distal pruning of pulmonary vessels seen on pulmonary angiography is also characteristic. Echocardiography, computed tomography, and magnetic resonance imaging scanning can allow for direct imaging of the tumor. Computed tomographic scanning cannot differentiate between thrombus and tumor; however, it can visualize secondary lesions. Gadolinium-diethylene triamene pentaacetic acid–enhanced magnetic resonance imaging may differentiate between vascularized tumor and intraluminal thrombus. Smith and colleagues [6] described magnetic and CT findings in pulmonary artery sarcoma, and they concluded that the tumor distribution of these lesions is highly distinctive and can be suggestive of the diagnosis. Preoperative histologic verification is generally not possible for vascular tumors; however, aspiration biopsy specimens may be obtained via pulmonary angioscopy, which allows direct visualization of the tumor.

The etiology of these tumors remains obscure. They have been related to the malignant degeneration of thrombus and the neoplastic transformation of primitive mesenchymal cells [7–9]. Grossly, these tumors are typically large and mucoid, and line or completely fill the pulmonary vessels but do not resemble thrombus (Fig 2). Morphologic descriptions of tumor growth patterns include intimal, intraluminal, and adventitial. The World Health Organization has set criteria for histologic classification of vascular sarcomas based on stained sections [10]. Immunohistochemical studies using smooth muscle actin, desmin, and S100 protein can assist in identifying the tumors. Positive staining for factor VII-related antigen and QBend/CD34 can suggest primary endothelial origin. Finally, Burke and Virmani [11] recently presented a clinicopathologic study of sarcomas of the great vessels and reported a wide variety of histologic findings.

View larger version (120K): [in this window] [in a new window]   Fig 2. . Specimens from right and left pulmonary arteries.

There were no cases of reperfusion edema after operation in this series. This is consistent with our experience in pulmonary thromboendarterectomy patients, in whom the incidence of clinically significant edema is now less than 10%.

The prognosis of these tumors depends largely on local recurrence. Length of survival of patients with primary pulmonary artery sarcomas is intermediate between that of patients with aortic and inferior vena caval sarcomas. Median length of survival without surgical resection is approximately 1.5 months, which is not affected by the addition of adjuvant therapy. Surgical resection has lengthened survival time to approximately 1 year. The addition of adjuvant radiotherapy and chemotherapy has extended this somewhat further. The survival times of our patients are consistent with these findings. The most beneficial protocols for this tumor, however, are yet to be described. Pulmonary metastases are present in up to 60% of patients, but extrathoracic disease is unusual. Thus, in the absence of distal disease, resection of pulmonary metastases after obtaining local control may provide a survival benefit. Distal microembolization is common; therefore, adjuvant chemotherapy, even in the absence of pulmonary nodules, would seem appropriate.

Our institution is involved actively in the evaluation and surgical therapy of patients with progressive pulmonary dysfunction related to chronic pulmonary thromboembolic disease, and we undoubtedly will continue to encounter primary pulmonary artery sarcomas in this endeavor. The extensive preoperative work-up currently used, perhaps with the addition of computed tomography and magnetic resonance imaging, should allow for earlier preoperative diagnosis. When clinicians encounter patients with advancing pulmonary failure who have an atypical history with findings suggestive of pulmonary artery occlusion, the diagnosis of pulmonary artery tumor must be considered. Emphasis should be placed on early identification of these lesions. As evidenced by our patients with an average duration of symptoms of 17 months, diagnosis is often delayed and the tumor is incurable. Total surgical resection with the addition of chemotherapy, radiotherapy, or both, or excision of all gross tumor in combination with adjuvant treatment should offer these patients significant palliation and an opportunity for increased length of survival.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comments References

 
Address reprint requests to Mr Jamieson, Division of Cardiothoracic Surgery, University of California Medical Center, 225 Dickinson St, San Diego, CA 92103–1990.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comments References

 

1. Jamieson SW, Auger WR, Fedullo PF, et al. Experience and results with 150 pulmonary thromboendarterectomy operations over a 29-month period. J Thorac Cardiovasc Surg 1993;106:116–26.[Abstract]
2. Becquemin JP, Lebbe C, Saada F, et al. Sarcoma of the aorta: report of a case and review of the literature. Ann Vasc Surg 1988;2:225–30.[Medline]
3. Delany SG, Doyle TCA, Bunton RW. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631–3.[Abstract/Free Full Text]
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5. Mandelstamm M. Uber primare Neubildugen des Herzens. Virchows Arch [Pathol Anat] 1923;245:43–54.
6. Smith WS, Lesar MS, Travis WD, et al. MR and CT findings in pulmonary artery sarcoma. J Comput Assist Tomogr 1989;13:906–9.[Medline]
7. Kattus AA, Longmire WP, Cannon JA, et al. Primary intraluminal tumor of the aorta producing malignant hypertension. N Engl J Med 1960;262:694–700.[Medline]
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10. Enzinger FM, Lattes R, Torloni R. Histological typing of soft tissue tumors: international classification of tumors. No 3. Geneva: WHO, 1969:19–25.
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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 Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Anderson, M. B. Articles by Jamieson, S. W. Search for Related Content PubMed PubMed Citation Articles by Anderson, M. B. Articles by Jamieson, S. W.