Ann Thorac Surg 2000;70:1416-1417
© 2000 The Society of Thoracic Surgeons
Case report
Papillary fibroelastoma of the left atrial appendage: echocardiographic findings
Takuro Tsukube, MDa,
Keiji Ataka, MDa,
Takahiro Taniguchi, MDb,
Mitsuhiro Yokoyama, MDb,
Keisuke Hanioka, MDc
a Department of Surgery, Division II, Kobe University, School of Medicine, Kobe, Japan
b First Department of Medicine, Kobe University, School of Medicine, Kobe, Japan
c Division of Surgical Pathology, Kobe University, School of Medicine, Kobe, Japan
Address reprint requests to Dr Tsukube, Department of Surgery, Division II, Kobe University, School of Medicine, 7-5-1 Kusunoki-cho, Chuou-ku, Kobe 650-0017, Japan
e-mail: ttsukube{at}med.kobe-u.ac.jp
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Abstract
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Papillary fibroelastoma is a small and rare benign intracardiac tumor that most frequently arises from the valvular endocardium. We report a patient with acute myocardial infarction in whom a papillary fibroelastoma in the left atrial appendage was detected by a transesophageal echocardiography during evaluation of the myocardial infarction. The roles of transesophageal echocardiography and surgical intervention are discussed.
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Introduction
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Papillary fibroelastoma is a small and rare benign intracardiac tumor that was formerly found primarily at autopsy. This lesion most frequently arises from the valvular endocardium and is usually asymptomatic [1]. Reports of surgical excision have become more frequent since the advent of transthoracic and transesophageal echocardiography (TEE). However, papillary fibroelastoma arising from the left atrium is still extremely rare and, to our knowledge, only 6 cases have previously been reported in the English literature [1, 2].
A 47-year-old man with no significant medical history was admitted on March 1, 1998, for acute myocardial infarction, with prominent clinical and echocardiographic evidence of left ventricular infarction. Coronary angiography demonstrated total occlusion of the midportion of the left anterior descending artery. Left ventriculography showed a large anteroseptal area of akinesis and an ejection fraction of 58%. Subsequently, percutaneous transluminal coronary angioplasty for the involved left anterior descending artery was performed successfully. During routine transesophageal echocardiography after coronary angioplasty, a pedunculated, mobile, and round tumor with a diameter of 15 mm and with a length of 70 mm was visualized in the left atrial appendage. It floated into the left ventricle through the mitral valve during diastole (Fig 1). No other tumors were observed in the cardiac cavities. Because of a predicted risk of recurrent embolization, surgical excision was performed on March 3, 1998.
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Fig 1. Transesophageal echocardiogram showing a pedunculated, mobile, and round tumor, with a diameter of 15 mm and a length of 70 mm, was visualized in the left atrial appendage. (LA = left atrium; LAA = left atrial appendage.)
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With bicaval and ascending aortic cannulation, total cardiopulmonary bypass was initiated. After cardioplegic arrest was achieved, the left atrium was opened. A yellow-white tumor with a smooth, rubbery surface was observed. The tumor was 15 mm in diameter and had a 70-mm long stalk attached to the left atrial appendage. After excision of the tumor arising from the left atrial appendage, and closure of the left atrium, the patient was weaned from cardiopulmonary bypass without difficulty. His postoperative course was uneventful. Histologic examination of the resected tumor showed the tip of the elongated fibrous tumor ramified into innumerable short branches imparting an appearance reminiscent of a sea anemone. Deposited fibrin and blood clot onto these processes obscured this typical gross feature. Microscopically, the tumor was comprised of fibrous tissue with an outer myxoid layer and a central fibroelastic core. The surface of the tumor was covered entirely with a layer of endocardial cells (Fig 2).
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Fig 2. (Top) Gross appearance of the tumor showing the approximately 70-mm long stalk, the tip of which appeared rounded in a club shape by virtue of a deposited fibrin clot. (Bottom) Microscopic view (H&E, x50) showing the ramified tip of the tumor. Each process was comprised of fibro-myxoid tissue lined by endocardial cells (left). Interspace of these processes was filled with fibrinous material (right).
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Comment
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Papillary fibroelastoma is the third most frequent benign tumor of the heart after myxoma and lipoma. Papillary fibroelastomas are derived from the endocardium and consist of the normal endocardial components, including fibrous tissue, elastic fibers, and smooth muscle cells. The typical papillary fibroelastoma resembles a sea anemone, with multiple papillary fronds attached to the endocardium by a short pedicle [1]. These tumors usually arise from the valvular endocardium, particularly that of the aortic valve. Only 10% are nonvalvular, and, to our knowledge, only 7 cases of papillary fibroelastoma of the left atrial atrium (including our patient), have been reported in the English literature [1, 2]. The vast majority of papillary fibroelastomas are incidentally detected at autopsy, or on surgically excised valves, and are not associated with cardiac symptoms. In recent years, these lesions have been diagnosed with increasing frequency by two-dimensional echocardiography [3]. Transesophageal echocardiography appears to be superior to transthoracic echocardiography for diagnosing these tumors and should be the preferred method for the preoperative evaluation of patients with valvular heart disease or suspected cardiac tumors.
It was initially thought that this tumor was not clinically significant, but there have been several recent reports of serious symptoms and complications that were attributed to tumor thromboembolism or myocardial ischemia. Whenever these tumors are found in patients with symptoms, surgery should be clearly recommended [4]. Even if it is an incidental finding, surgical excision is indicated in all patients, regardless of the site of origin, because left-sided lesions have been shown to cause cardiac and cerebral symptoms, and fatal embolism can be induced even from the right side of the heart [5].
Reports of left atrial papillary fibroelastoma are extremely rare but such lesions are associated with a high risk of cerebral embolism and myocardial infarction. Among the seven reported tumors, two were associated with transient ischemic attacks and our patient had acute myocardial infarction. As surgical excision is curative and no recurrence has been reported, a left atrial papillary fibroelastoma should be removed, even in an asymptomatic patient, to prevent catastrophic embolic complications.
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References
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Burke A., Virmani R. Papillary fibroelastoma. In: Burke A., Virmani R., eds. Tumors of the heart and great vessels. Third series, fascicle 16. Washington, DC: Armed Forces Institute of Pathology, 1996:47-54.
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Klarich K.W., Enriquez-Sarano M., Gura G.M., Edwards W.D., Tajik A.J., Seward J.B. Papillary fibroelastoma. J Am Coll Cardiol 1997;30:784-790.[Abstract]
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Hicks K.A., Kovach J.A., Frishberg D.P., Wiley T.M., Gurczak P.B., Vernalis M.N. Echocardiographic evaluation of papillary fibroelastoma. J Am Soc Echocardiogr 1996;9:353-360.[Medline]
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Valente M., Basso C., Thiene G., et al. Fibroelastic papilloma. Cardiovasc Pathol 1992;1:161-166.
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Shahian D.M., Labib S.B., Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59:538-541.[Abstract/Free Full Text]
Accepted for publication January 17, 2000.
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Abstract
Introduction
