Ann Thorac Surg 2008;86:297-299. doi:10.1016/j.athoracsur.2008.01.068
© 2008 The Society of Thoracic Surgeons
Case Reports
Chondrosarcoma Masquerading as Cardiomyopathy
Charles R. Mulligan, Jr, MAJ, MC*,
Houman Tavaf-Motamen, LTC, MC,
Robert Stewart, LTC, MC,
William C. Devries, COL, MC*
Division of Cardiothoracic Surgery, Walter Reed Army Medical Center, Washington, DC
Accepted for publication January 21, 2008.
* Address correspondence to Dr Mulligan, Division of Cardiothoracic Surgery, Rm 4655, Department of Surgery, Walter Reed Army Medical Center, 6900 Georgia Ave, NW, Washington, DC 20307-5001 (Email: charles.mulligan{at}us.army.mil).
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Abstract
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A 17-year-old male patient presented to his primary care provider with heart failure symptoms and was transferred to our hospital with the diagnosis of idiopathic cardiomyopathy. His workup identified a large mediastinal mass with right ventricular outflow obstruction, which was resected. The pathology of the mass was a low-grade chondrosarcoma. The patient currently remains disease free at 4 years.
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Introduction
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Chondrosarcomas are the most common primary chest wall malignant tumors. They generally present with an enlarging, painful, chest wall mass. We present an unusual case of a chondrosarcoma presenting with symptoms related to cardiac compression.
A 17-year-old male patient presented to his primary care provider with a 2-month history of cough, congestion, and a 50-pound weight loss. He also reported dyspnea at rest, 2-pillow orthopnea, and general fatigue. He denied any fever, chills, or night sweats. He denied any chest pain or paroxysmal nocturnal dyspnea. The physical examination revealed bilateral wheezing, distant heart sounds without murmur, and a faint gallop.
A chest roentgenogram revealed cardiomegaly, with no evidence of pleural effusion (Fig 1). An electrocardiogram demonstrated low voltage and a sinus tachycardia. A transthoracic echocardiogram was limited due to poor windows but found a moderately sized pericardial effusion, without evidence of hemodynamic compromise, and a dilated right atrium and ventricle with mild tricuspid regurgitation. A subsequent computed tomography (CT) scan demonstrated a large anterior mediastinal mass compressing the trachea and right ventricular outflow tract (RVOT; Fig 2).
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Fig 2. A chest computed tomography scan demonstrates extrinsic compression of the right ventricular out flow tract with posterior cardiac displacement.
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In the operating theater, an awake, fiberoptic double-lumen tube was placed, and he underwent an en bloc sternectomy and chest wall resection. The tumor had not invaded the pericardium or the heart. The patient experienced transient right heart dysfunction upon removal of the outflow obstruction, which responded to dobutamine.
The anterior chest wall was reconstructed with Dualmesh (W. L. Gore & Associates, Flagstaff, AZ) and methylmethacrylate and was covered with pectoralis major flaps. Histopathology revealed a low-grade chondrosarcoma, 17.0 x 15.0 x 14.0 cm, without invasion of anterior ribs or sternum (Fig 3). He has been followed up with serial chest CT scans and remains disease free at 4 years.
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Comment
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Chondrosarcoma is the most common primary tumor of the chest wall. It generally presents as a painful, slowly enlarging chest wall mass. Chondrosarcomas rarely present with manifestations of cardiac compression. Castillo and colleagues [1] published the only case report in the English literature of extrinsic cardiac compression from chondrosarcoma in 1966.
Although uncommon, other neoplasms can present with symptoms of cardiac compression. Shaver and colleagues [2] presented a patient with Hodgkin disease of the thymus that caused extrinsic compression of the RVOT. Their review of the literature found 7 additional case studies of cardiac compression, with 3 caused by teratomas, 2 by lymphomas, 1 pericardial mesothelioma, and 1 lymphatic cyst [2]. Marshall and colleagues [3] found 35 case studies, predominantly teratomas and Hodgkin disease, causing extrinsic RVOT obstruction. More recent literature examining the benefit of echocardiography in the diagnosis of mediastinal masses revealed additional cases of teratoma and lymphoma causing extrinsic compression of the RVOT [4–6].
The management of this and all patients presenting with tumors compressing the heart requires a thoughtful and multidisciplinary approach from diagnosis, to anesthetic management, to surgical approach and subsequent medical management.
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Footnotes
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* The views expressed in this article are the views of the authors and should not be construed as official or as reflecting the policies of the Department, of the Army or Department of Defense. 
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References
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- Del Castillo JJ, Gianfrancesco H, Mannix Jr EP. Pulmonic stenosis due to extrinsic compression by sternal chondrosarcoma J Thorac Cadiovasc Surg 1966;52:255-260.[Medline]
- Shaver VC, Bailey Jr WR, Marrangoni AG. Acquired pulmonic stenosis due to external cardiac compression Am J Cardiol 1965;16:256-261.[Medline]
- Marshall ME, Trump DL. Acquired extrinsic pulmonic stenosis caused by mediastinal tumors Cancer 1982;49:1496-1499.[Medline]
- Canedo MI, Otken L, Stefadouros MA. Echocardiographic features of cardiac compression by a thymoma simulating cardiac tamponade and obstruction of the superior vena cava Br Heart J 1977;39:1038-1042.[Abstract/Free Full Text]
- Baduini G, Paolillo V, Di Summa M. Echocardiographic findings in a case of acquired pulmonic stenosis from extrinsic compression by a mediastinal cyst Chest 1981;80:507-509.[Medline]
- Mandysova E, Neuzil P, Niederle P, Belohlavek O, Kozac T, Mandys V. Pulmonary stenosis caused by extrinsic compression of non-Hodgkin's lymphoma Echocardiography 2004;21:564-567.
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Abstract
Introduction
