HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

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): Jules E. Dussek David R. Anderson Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Akomea-Agyin, C. Articles by Hartley, R. B. Search for Related Content PubMed PubMed Citation Articles by Akomea-Agyin, C. Articles by Hartley, R. B.

Ann Thorac Surg 1996;61:1536-1538
© 1996 The Society of Thoracic Surgeons

---

Case Report

Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism: Successful Surgical Intervention

Departments of Cardiothoracic Surgery and Histopathology, United Medical and Dental School, Guy's Hospital, London, United Kingdom

Accepted for publication November 28, 1995.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
Because of its rarity and the similarity of its presentation to that of pulmonary thromboembolic disease, the diagnosis of pulmonary artery sarcoma is often not considered early in patients presenting with recurrent or chronic pulmonary emboli. We present a case of pulmonary artery sarcoma that was treated as pulmonary embolism for 3 years before surgical resection was carried out. Two years after the resection the patient is well with no clinical or radiologic evidence of recurrent or metastatic disease.

	Introduction

Top Footnotes Abstract Introduction Comment References

 
Primary sarcomas of the pulmonary artery are rare. About 120 cases have been reported since it was first described in 1923 by Mandelstamm [1]. The prognosis is poor; the median survival after resection and tissue diagnosis is 10 months [2]. The in vivo rate of growth of the tumor is unknown, but it can be rapid [3], with patients dying of right heart failure or metastases. Burke and Virmani [4], however, in their clinicopathologic study of sarcomas of the great vessels, have reported a mean survival of 23 months for pulmonary artery sarcomas, being intermediate between that of aortic sarcomas and those arising in the inferior vena cava. In this case report the diagnosis of pulmonary artery sarcoma was made 3 years after the initial presentation and the patient has survived 2 years after complete resection, with no clinical or radiologic evidence of recurrent or metastatic disease.

A 30-year-old woman presented to her local hospital in May 1990 with a 3-day history of hemoptysis and pleuritic left-sided pain. She was a known asthmatic and had suffered a spontaneous pneumothorax at the age of 20 years.

Examination revealed a 3/6 ejection systolic murmur, loudest at the left sternal border and without radiation. There were no other abnormal physical signs. A chest radiograph showed a small left pleural effusion, and a ventilation/perfusion scan demonstrated a perfusion defect in the left base. There was no electrocardiographic evidence of right ventricular hypertrophy. Bilateral venograms failed to show any evidence of deep venous thrombosis, and pulmonary arteriography showed an occluded left lower lobe artery. A diagnosis of acute pulmonary embolism of uncertain source was assumed, and she was anticoagulated for 6 months.

Two weeks after stopping warfarin administration, she suffered a further episode of hemoptysis, and a ventilation/perfusion scan demonstrated persistence of the perfusion defect to the left lower zone. Again bilateral venograms failed to show any evidence of deep venous thrombosis, and pelvic ultrasound was normal. It was decided to reinstate anticoagulation for 2 years. During this period, she suffered progressive dypsnea on exertion but no significant acute symptoms.

A repeat ventilation/perfusion scan in October 1992 before the planned discontinuation of anticoagulation surprisingly showed no perfusion of the left lung, and pulmonary arteriography showed total occlusion of the left pulmonary artery (Fig 1). It was assumed that further thromboembolism had occurred despite anticoagulation, and local streptokinase infusions over 24 hours were carried out. This failed to lyse the ``clot,'' and the follow-up films showed an intraluminal filling defect in the left pulmonary artery. She was therefore referred for further investigation. Magnetic resonance imaging using gadolinium-diethylenetriamine pentaacetic acid showed a vascularized pulmonary artery tumor, probably a sarcoma (Fig 2). A number of small opacities were present in the periphery of both lungs. Two-dimensional echocardiography confirmed a mass in the main pulmonary artery with occlusion of the left pulmonary artery.

View larger version (154K): [in this window] [in a new window]   Fig 1. . Pulmonary arteriogram showing total occlusion of left pulmonary artery with complete absence of vascular markings in the whole of the left lung.

View larger version (165K): [in this window] [in a new window]   Fig 2. . Magnetic resonance imaging (gadolinium-diethylenetriamine pentaacetic acid) showing an enhancing tumor in the main pulmonary artery (arrow) with extension into both the right and left pulmonary arteries.

Sections of the intraarterial lesion showed it was continuous with the intimal layer of the pulmonary artery. There were solid areas composed of plump histiocytes and spindle cells with variable amounts of collagen (Fig 3). Only scanty mitotic figures were identified. There was no evidence of extension of the lesions beyond the arterial wall, and the resection edge was free of tumor. Adjacent lymph nodes showed follicular hyperplasia only. These appearances are most unusual at this site, but are regarded as a low-grade intimal sarcoma, the exact histogenesis of which is uncertain. The parencyhmal lung lesions consisted of areas of fibrosis with scattered hemosiderin-laden macrophages, chronic inflammatory cells, and eosinophils. There was pneumocyte hyperplasia and fibrous intimal thickening of small arteries, and one vessel contained a nodule of the primary lesion, without evidence of infiltration. Elsewhere appearances were not similar to those of the pulmonary arterial lesion, and were interpreted as representing organized infarcts.

View larger version (119K): [in this window] [in a new window]   Fig 3. . Micrograph from a cellular area of the tumor showing plump spindle cells with a small amount of collagenous stroma. (Hematoxylin and eosin; x400 before 57% reduction.)

	Comment

Top Footnotes Abstract Introduction Comment References

 
Pulmonary artery sarcomas arise from the intimal or subintimal areas of the pulmonary artery and are hypothesized to arise from primitive cells of the bulbus cordis [5]. They generally spread in the direction of flow. Rarely they spread in retrograde fashion and, as demonstrated in the case report, this low-grade sarcoma first occurred in the left lower lobe artery and spread retrogradely into the pulmonary trunk, occluding the left pulmonary artery over a period of 3 years.

The diagnosis of pulmonary sarcoma is difficult because the symptoms are insidious, nonspecific, and indistinguishable from those of pulmonary thromboembolic disease. In a review of 110 cases of pulmonary artery sarcoma Nanomuna and associates [6] reported the most common symptoms as dyspnea (67%), chest pain (54%), cough (43%), and hemoptysis (22%). Patients with chronic pulmonary embolism also suffer from progressive exertional dypsnea and chest pain punctuated by episodes of hemoptysis from ruptured bronchial collaterals [7]. This patient's presenting and recurrent symptoms of hemoptysis and pleuritic pain are those of pulmonary infarction, which she clearly had from the histology of the resected left lung.

Diagnostic investigations start with a plain chest radiograph, which may be reported as normal. In chronic pulmonary embolism pulmonary oligemia is present in more than 50% of cases [7].

Pulmonary arteriogram shows intraluminal failing defects but cannot distinguish between tumor and thrombus. The multiplanar capabilities of magnetic resonance imaging and spiral computed tomographic scanning make them ideal for showing the extent of these intra-vascular lesions and with contrast enhancement can show their nature as well. However, by not using ionizing radiation, gadolinium-diethylenetriamine pentaacetic acid magnetic resonance imaging has an advantage over computed tomographic scanning and has become a useful diagnostic tool for this rare tumor [8].

Distal pulmonary tumor emboli are frequently seen in sarcomas of the pulmonary artery, occurring in two-thirds of reported cases. In this report the intravascular tumor nodule seen in the parenchymal lung section showed no evidence of infiltration. The microscopic picture of the primary tumor was that of an undifferentiated intimal sarcoma. This is in keeping with one-third of reported cases, the remainder showing variable cellular differentiation (myogenic, chondro-osseous, fibroblastic, or endothelial). Unfortunately neither the degree of mesenchymal differentiation nor the cytologic grade of the tumor has been reported to be of prognostic significance.

Early diagnosis and surgical resection remain the mainstay of treatment. There is no good evidence that chemotherapy and radiotherapy have any benefits, although a case of improved survival has been reported when used as adjuvant therapy after excision of an advanced tumor [2]. Increased clinical awareness of this unusual tumor, especially in patients presenting with features of recurrent or unilateral pulmonary emboli or who appear to extend their emboli while under full anticoagulation, will lead to more of these cases being correctly diagnosed early.

	Footnotes

Top Footnotes Abstract Introduction Comment References

 
Address reprint requests to Mr Dussek, Department of Cardiothoracic Surgery, Guy's Hospital, St Thomas' St, London SE1 9RT, United Kingdom.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Mandelstamm M. Ueber primare Neubildungen des Herzens. Virchows Arch [A] 1923;245:43–54.
2. Head HD, Flam MS, John MJ, Lipnik SS, Slater DL, Stewart RD. Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy. Ann Thorac Surg 1992;53:332–4.[Abstract/Free Full Text]
3. Madu EC, Taylor DC, Durzinsky DS, Fraker TD. Primary intimal sarcoma of the pulmonary trunk simulating pulmonary embolism. Am Heart J 1993;125:1790–2.[Medline]
4. Burke AP, Virmani R. A clinicopathologic study. Sarcomas of the great vessels. Cancer 1993;71:1761–73.[Medline]
5. Bleisch VR, Kraus FT. Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with liptomeric organelles and ultrastructural features of rhabdomyosarcoma. Cancer 1980;46:314–24.[Medline]
6. Nanomuna A, Kurumay H, Kono N, et al. Primary pulmonary artery sarcoma. Report of two cases and studied by immunohistochemistry and electric microscopy and a review of 110 cases in the literature. Acta Pathol Jpn 1988;38:883–96.[Medline]
7. Chitwood WR Jr, Lyerly HK, Sabiston DC Jr. Surgical management of chronic pulmonary embolism. Ann Surg 1985;201:11–26.[Medline]
8. Sato H, Matsuda H, Hatakeyama T, Okada M, Nakamura K. Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment. J Cardiovasc Surg 1992;33:202–3.[Medline]

This article has been cited by other articles:

				J. S. Park, J.-H. Chung, S. Jheon, D.-J. Choi, H. I. Yoon, J. H. Lee, C.-T. Lee, and S. W. Lee EBUS-TBNA in the differential diagnosis of pulmonary artery sarcoma and thromboembolism Eur. Respir. J., December 1, 2011; 38(6): 1480 - 1482. [Full Text] [PDF]

				J. Shehatha, P. Saxena, B. Clarke, J. Dunning, and I. E. Konstantinov Surgical Management of Extensive Pulmonary Artery Sarcoma Ann. Thorac. Surg., April 1, 2009; 87(4): 1269 - 1271. [Abstract] [Full Text] [PDF]

				S. H. Blackmon, D. C. Rice, A. M. Correa, R. Mehran, J. B. Putnam, W. R. Smythe, J.-C. Walkes, G. L. Walsh, C. Moran, H. Singh, et al. Management of Primary Pulmonary Artery Sarcomas Ann. Thorac. Surg., March 1, 2009; 87(3): 977 - 984. [Abstract] [Full Text] [PDF]

				G. Shanmugam Primary cardiac sarcoma Eur J Cardiothorac Surg, June 1, 2006; 29(6): 925 - 932. [Full Text] [PDF]

				A. Mattoo, P. F. Fedullo, D. Kapelanski, and J. S. Ilowite Pulmonary Artery Sarcoma* : A Case Report of Surgical Cure and 5-Year Follow-up Chest, August 1, 2002; 122(2): 745 - 747. [Abstract] [Full Text] [PDF]

				C. J. Bergin, J. P. Hauschildt, M. A. Brown, R. N. Channick, and P. F. Fedullo Identifying the Cause of Unilateral Hypoperfusion in Patients Suspected to Have Chronic Pulmonary Thromboembolism: Diagnostic Accuracy of Helical CT and Conventional Angiography Radiology, December 1, 1999; 213(3): 743 - 749. [Abstract] [Full Text]

				P. Dumont, P. Diot, M. R. Aupart, and B. Toumieux Leiomyosarcoma of the pulmonary artery Ann. Thorac. Surg., December 1, 1998; 66(6): 2089 - 2091. [Abstract] [Full Text] [PDF]

				G. B. Luciani, A. Mazzucco, J. E. Dussek, and C. Akomea-Agyin Surgical Management of Primary Pulmonary Artery Sarcoma Ann. Thorac. Surg., February 1, 1997; 63(2): 601 - 602. [Full Text]

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): Jules E. Dussek David R. Anderson Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Akomea-Agyin, C. Articles by Hartley, R. B. Search for Related Content PubMed PubMed Citation Articles by Akomea-Agyin, C. Articles by Hartley, R. B.