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Ann Thorac Surg 2009;87:1269-1271. doi:10.1016/j.athoracsur.2008.08.030
© 2009 The Society of Thoracic Surgeons
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Case Reports

Surgical Management of Extensive Pulmonary Artery Sarcoma

Jaffar Shehatha, FRCS, FRACSa, Pankaj Saxena, MCh, DNBa, Belinda Clarke, FRCPA, PhDb, John Dunning, FRCSa, Igor E. Konstantinov, MD, PhDa,c,*

a Department of Cardiothoracic Surgery, The Prince Charles Hospital, Chermside, Queensland, Australia
b Department of Anatomical Pathology, The Prince Charles Hospital, Chermside, Queensland, Australia
c University of Western Australia, Perth, Western Australia, Australia

Accepted for publication August 13, 2008.

* Address correspondence to Prof Konstantinov, University of Western Australia, Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, G Block, 6th Floor, Hospital Ave, Nedlands, WA, Australia (Email: konstantinov.igor{at}alumni.mayo.edu).


   Abstract
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Primary pulmonary artery sarcoma is a rare tumor that can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. This article reports a patient with a preoperative diagnosis of pulmonary embolism who was found to have an extensive pulmonary artery tumor. Surgical resection of the primary pulmonary artery sarcoma and reconstruction of the central pulmonary arteries, followed by adjuvant chemoradiotherapy, provided significant improvement in his clinical symptoms.


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Primary pulmonary artery (PA) sarcoma is a rare tumor. It is often misdiagnosed as chronic pulmonary thromboembolic disease [1]. We report a patient with a preoperative diagnosis of pulmonary embolism who was found to have an extensive PA tumor intraoperatively. Surgical resection of his primary PA sarcoma and reconstruction of the central PAs, followed by adjuvant chemoradiotherapy, provided significant improvement from debilitating symptoms.

A 61-year-old man presented with dyspnea on exertion and was clinically diagnosed as having a pulmonary embolism. The patient was anticoagulated with heparin and was referred to us because he failed to respond to medical therapy. The result of the clinical examination was normal.

The patient underwent further tests because of equivocal findings on a computed tomographic (CT) scan. A transthoracic echocardiogram revealed normal systolic function of both ventricles. A second CT scan revealed multiple filling defects in the central PAs and total occlusion of the right PA (Fig 1).


Figure 1
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  		Fig 1. (A, B) Computed tomographic scan demonstrates an extensive filling defect of the main, right, and left pulmonary arteries. The right pulmonary artery is completely occluded with an intraluminal tumor.

 
An inferior vena caval filter was inserted percutaneously. A coronary angiogram demonstrated minor coronary artery disease. A median sternotomy was performed, and cardiopulmonary bypass was established using bicaval cannulation. The patient was cooled to 18°C. Antegrade cold crystalloid cardioplegia was delivered for myocardial protection.

Extensive tumor involving the main PA, pulmonary valve, and the left and right PAs was found after an extended pulmonary arteriotomy. Exposure of right PA was facilitated by transecting the ascending aorta. A proper plane was developed between the tumor and the wall of the PA. Bilateral tumor thrombectomy was performed. The tumor was resected en bloc along with the pulmonary valve and main PA. The main PA was reconstructed using a pulmonary homograft that was anastomosed distally to the confluence of the PAs and proximally to the right ventricular outflow tract. The ascending aorta was repaired in a standard fashion.

The patient was weaned from cardiopulmonary bypass with minimal inotropic support. He had an uneventful postoperative course.

The histopathologic features of the tumor were those of a pleomorphic malignancy comprising spindled and epithelioid cells admixed with inflammatory cells and fibrin in a loose myxoid stroma. There were areas of necrosis and frequent mitoses (Fig 2). The tumor predominantly involved the intima with focal invasion of the media, but there was no involvement of the arterial adventitia or of the myocardium of right ventricular outflow tract. These features were consistent with a diagnosis of primary PA sarcoma.


Figure 2
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  		Fig 2. (A) Polypoid luminal aspect of pulmonary artery sarcoma (hematoxylin and eosin, original magnification x20). (B) Pleomorphic spindled and epithelioid tumour cells admixed with fibrin (hematoxylin and eosin, original magnification x100).

 
Postoperatively the patient was not found to have any distant metastatic disease. The patient was treated with adjuvant chemotherapy with Adriamycin (Pharmacia S.p.A, Milan, Italy) and ifosfamide and 10 cycles of radiotherapy. The patient died 3 years later after development of metastatic disease to the brain and adrenal glands.


   Comment
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References
 
Acute pulmonary embolism and chronic thromboembolic pulmonary disease are often under-diagnosed. Primary PA sarcoma is a rare tumor that can mimic these conditions. This tumor causes bulky mucoid growth that usually originates from and grows along the intima of the arterial wall in the direction of blood flow [2]. It may partially or sometimes totally occlude the main PA and branch PAs.

Diagnosis of this tumor is often difficult and late. In retrospect, the protuberances of the mass and its pattern in the main PA on the CT scan in our patient were not typical of thrombosis. However, owing to the rarity of the PA tumors, a high index of suspicion is needed to establish a preoperative diagnosis. The definitive diagnosis in most clinical scenarios is established after surgical excision. Patients with PA sarcoma commonly present with atypical symptoms or symptoms suggestive of chronic pulmonary thromboembolism. There is a progressive pulmonary dysfunction and right ventricular failure due to pulmonary hypertension. Differentiation between pulmonary embolism and primary PA sarcoma preoperatively can be difficult, as evident from our experience. Contrast enhancement of an intraluminal filling defect in the PA and sometimes demonstration of extravascular spread of the tumor may lead to suspicion of primary PA sarcoma [3].

The natural history of this condition may be unpredictable. Some patients may be treated medically for long time and then referred for surgical intervention after failure to respond to medical therapy [4]. Others may have a downhill course, with death due to progressive pulmonary hypertension causing congestive heart failure or progression of metastatic disease.

Early diagnosis and aggressive surgical resection in operable patients may significantly improve clinical symptoms. Adjuvant chemotherapy and radiotherapy has been found to provide prolonged tumor-free survival [5].

In summary, primary PA sarcoma can present to the cardiothoracic surgeon as a pulmonary embolism or as chronic thromboembolic pulmonary disease for surgical treatment. Surgical resection of even locally advanced malignancy with reconstruction of PAs using pulmonary homograft may provide satisfactory clinical outcome. A high index of suspicion is required to establish an early diagnosis.


   References
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 Abstract
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 Comment
 References
 

  1. Hoeper MM, Mayer E, Simmoneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension Circulation 2006;113:2011-2020.[Free Full Text]
  2. Sebenik M, Ricci Jr A, DiPasquale B, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature Am J Surg Pathol 2005;29:1184-1193.[Medline]
  3. Scheffel H, Stolzmann P, Plass A, et al. Primary intimal pulmonary artery sarcoma: a diagnostic challenge J Thorac Cardiovasc Surg 2008;135:949-950.[Free Full Text]
  4. Akomea-Agyin C, Dussek JE, Anderson DR, Hartley RB. Pulmonary artery sarcoma mimicking pulmonary embolism: successful surgical intervention Ann Thorac Surg 1996;61:1536-1538.[Abstract/Free Full Text]
  5. 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;53:332-334.[Abstract/Free Full Text]



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[Abstract] [Full Text] [PDF]

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