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J Thorac Cardiovasc Surg 2004;128:763-765
© 2004 The American Association for Thoracic Surgery

Brief communication

Failure of medical therapy for pulmonary "thromboembolic" disease: Beware the unsuspected primary sarcoma of the pulmonary artery

^a Department of Surgery, Division of Cardiothoracic Surgery, Washington University School of Medicine, St Louis, Mo, USA

Received for publication March 15, 2004; accepted for publication March 30, 2004.

^* Address for reprints: Jennifer S. Lawton, MD, Department of Surgery, Division of Cardiothoracic Surgery, Washington University School of Medicine, Queeny Tower, Suite 3108, One Barnes-Jewish Hospital Plaza, St Louis, MO 63110, USA
lawtonj@msnotes.wustl.edu

The first 20% of the full text of this article appears below.

Primary sarcoma of the pulmonary artery (PA) is rare. The diagnosis is difficult and often delayed. Two patients with primary sarcoma of the PA that mimicked pulmonary thromboembolic diseases who presented with complications requiring surgical intervention are described. The diagnostic and management issues regarding these often unsuspected but aggressive tumors are discussed.

Clinical summary

PATIENT 1. A 54-year-old woman was admitted for pulmonary thromboendarterectomy workup. She had a past medical history of pulmonary embolism (PE) that occurred 3 months before admission. This was complicated by an area of right lower lobe infarction seen on chest computed tomography (CT) scanning. A deep vein thrombosis of her right femoral vein was diagnosed on lower extremity Doppler investigation as the cause of her PE. Her treatment consisted of anticoagulation and placement of an inferior vena cava filter. The results of all screening tests for a hypercoagulable state were negative. She had progressive shortness of breath over the following 3-month period and was in New York Heart Association class IV at the time of admission. A chest CT scan revealed a massive pulmonary embolus in the right PA (Figure 1, A... A pulmonary arteriogram demonstrated total occlusion of the right PA approximately 4 cm from its origin, whereas the left PA and its branch vessels were normal (Figure 1, B... Echocardiography revealed severe tricuspid regurgitation and a PA systolic pressure of 65 mm Hg. In view of her symptoms and occluded right PA, she underwent a right pulmonary endarterectomy to restore antegrade right pulmonary blood flow. Intraoperatively, extensive clots within the right PA were . . . [Full Text of this Article]