(Circulation. 2000;101:2220.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
From Cardiology, Division of Medicine (M.Y., S.F., K.M.), Division of Cardiovascular Surgery (K.B.), and Division of Pathology (H.I.U., C.Y.), National Cardiovascular Center, Osaka, Japan.
Correspondence to Masakazu Yamagishi, MD, FACC, Cardiology, Division of Medicine, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565, Japan. E-mail myamagi{at}hsp.ncvc.go.jp
A 30-year-old
woman was referred to our clinic because of shortness of breath and
general fatigue. She had been well until 2 months earlier, when she
noticed mild shortness of breath during and after exercise. Physical
examination revealed a systolic heart murmur at the left
parasternal area. Transthoracic
echocardiography demonstrated an abnormal mass
located just proximal to the pulmonary valve occupying the
right ventricular outflow tract (Figure 1
, top left). There was a
small pericardial effusion, and a flattened ventricular
septum suggested elevated right ventricular pressure. The
regionally magnified image indicated relatively low echogenicity within
the mass (Figure 1
, top right). This mass was attached to the
side of the right ventricular free wall and was free from
the pulmonary valve. Transesophageal
echocardiography demonstrated the mass in the right
ventricular outflow tract with accelerated color flow
across the mass, suggesting hemodynamically significant
obstruction (Figure 1
, bottom). Ultrasound and
radiographic examinations did not demonstrate any other
primary lesions in the abdominal or genital organs. One week later,
radical resection of the mass and reconstruction of the right
ventricular outflow tract were performed. The tumor
originated from the right ventricular outflow tract. A
small part of it had extended beyond the pulmonary valve into
the main pulmonary artery. The mass seemed to be a myxoma
(Figure 2
, left), but
microscopic examination of hematoxylin-eosinstained specimens showed
a uniform population of large atypical cells with prominent nucleoli.
Interestingly, there was evidence of an osteogenic sarcomatous element
(Figure 2
, right). Immunohistochemical stains were negative for
such antigens as myoglobin,
-actin, S-100, factor VIII, CD31, and
CD34. Thus, the tumor was considered to be an osteosarcoma, which
constitutes only 0% to 3.4% of cardiac tumors.1 2
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Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Lukes Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Lukes Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
References
1. Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg. 1991;51:906910.[Abstract]
2. Burke A, Virmani R. Primary cardiac sarcomas. In: Atlas of Tumor Pathology: Tumors of the Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology; 1996:127170.
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