Circulation. 2001;103:e99-e100
(Circulation. 2001;103:e99.)
© 2001 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Syncope in a Patient With Spindle Cell Sarcoma of the Main Pulmonary Artery
Bernardo Treistman, MD;
Robert J. Card, MD;
Tomas Klima, MD;
Scott Flamm, MD
From the Department of Cardiology, St Lukes Episcopal Hospital,
Texas Heart Institute, Houston, Tex.
Correspondence to Bernardo Treistman, MD, St Lukes Medical Towers, 6624 Fannin, Suite 2590, Houston, TX 77030.
A 44-year-old man was hospitalized after
a syncopal episode. A thrill and loud systolic ejection murmur
were present over the left border of the sternum. A 12-lead ECG and
chest x-rays were normal. A 2D echocardiogram and Doppler studies
documented normal cardiac chambers and a peak systolic
pulmonary transvalvular gradient of 80 mm Hg.
Biplane right ventriculography revealed a large mobile mass in the main
pulmonary artery extending into the right ventricular outflow tract
(Figure 1
). Cardiac MRI showed the mass within the same area (Figure 2
and Figure
I, which can be found at www.circulationaha.org). The patient underwent surgical resection of a large tumor in the main pulmonary artery (Figure 3
). The tumor extended into the right pulmonary artery. The pulmonary valve was replaced with a homograft, and an endarterectomy of the main and
right pulmonary arteries was accomplished. Histological examination revealed a spindle cell sarcoma
(Figures 4
and 5
). The patient remained asymptomatic and had no cardiac murmurs 8 months after surgery.1

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Figure 1. Biplane right ventriculography reveals a large mobile mass in the main pulmonary artery that extends into the right ventricular outflow tract (arrow).
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Figure 2. MRI taken before (left) and after (right) administration of gadolinium. Images were obtained in the right ventricular outflow tract projection. A large, enhancing mass (arrows) is present in the main pulmonary artery and upper outflow tract.
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Figure 4. The tumor shows features of highly pleomorphic cell malignant neoplasm, with numerous mitotic figures, some of which are atypical (arrow). Movats Pentachrome stain, x360.
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Figure 5. The tumor occupies the lumen of the pulmonary artery and does not invade its wall. The internal elastic lamina (arrow) is not interrupted. Movats Pentachrome stain, x27.
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Footnotes
Figure
I is a cine image and can be found Online at www.circulationaha.org.
References
1.
Bleisch N, Kraus F. Polypoid sarcoma of the pulmonary trunk.
Cancer. 1980;46:314.[Medline]
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