Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences Pulmonary Artery Sarcoma Masquerading as Pulmonary Embolism Olurotimi Adekolu1, Justin Lundbye1, and Constantine Manthous2,3 1

Department of Medicine, University of Connecticut, Farmington, Connecticut; 2Department of Medicine, Yale University, New Haven, Connecticut; and 3Department of Medicine, Hospital of Central Connecticut, New Britain, Connecticut

A 58-year-old man presented with 2 months of cough, dyspnea on exertion, and progressive bilateral lower extremity edema. Computed tomography angiogram showed a filling defect larger than 5 cm occluding both pulmonary arteries (Figure 1). He was treated expectantly with anticoagulants for pulmonary embolism but returned 2 weeks later with worsening dyspnea and echocardiographic evidence of right heart failure. Positron emission tomography scan showed moderate-to-intense uptake of the left main and lobar pulmonary artery corresponding to perfusion defects of tomograms (Figure 2). Surgical thromboendarterectomy revealed a high-grade myxoid spindle cell sarcoma. Pulmonary artery sarcomas are rare tumors arising from the mesenchymal cells of the intima of blood vessels that may mimic pulmonary embolism. Positron emission tomography scanning can distinguish pulmonary embolism from pulmonary artery sarcoma (1), for which the prognosis is generally very poor (2, 3).

Figure 1.

Figure 2.

Author disclosures are available with the text of this article at www.atsjournals.org.

References 1. Ito K, Kubota K, Morooka M, Shida Y, Hasuo K, Endo H, Matsuda H. Diagnostic usefulness of 18F-FDG PET/CT in the differentiation of pulmonary artery sarcoma and pulmonary embolism. Ann Nucl Med 2009;23:671–676.

Am J Respir Crit Care Med Vol 188, Iss. 9, p 1161, Nov 1, 2013 Copyright ª 2013 by the American Thoracic Society DOI: 10.1164/rccm.201304-0715IM Internet address: www.atsjournals.org

2. Krüger I, Borowski 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;38:91–95. 3. Hu XP, Xu JP, Liu NN. Primary pulmonary artery sarcoma: surgical management and differential diagnosis with pulmonary embolism and pulmonary valve stenosis. J Card Surg 2009;24:613–616.

Pulmonary artery sarcoma masquerading as pulmonary embolism.

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