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Papillary fibroelastoma of the left atrial appendage
Papillary Fibroelastoma of the Left Atrial Appendage J. S. Sidhu, MB, BS, MRCP, M. Harries, MD, FRCP, and R. Senior, MD, DM, FRCP(Lond), Middlesex, United Kingdom
Cardiac papillary fibroelastomas are benign tumors that usually arise from the valvular endocardium. They are clinically important because of their propensity to embolize. We describe, to the best of our knowledge, the first reported case of a papillary fi-
broelastoma arising from the left atrial appendage, giving rise to multiple cerebral embolic events. The tumor was excised surgically, with no further embolic events. (J Am Soc Echocardiogr 2001;14:838-9.)
CASE REPORT
DISCUSSION
Papillary fibroelastomas are rare, benign tumors that arise almost exclusively from valvular endocardium.They have a well-known potential for causing systemic embolization.1 We describe, to the best of our knowledge, the first reported case of a papillary fibroelastoma of the left atrial appendage.The tumor was the cause of recurrent cerebral emboli and was successfully excised. A 59-year-old, right-handed woman had sudden weakness of the left arm and left leg. She had no history of cardiac symptoms or any risk factors for cerebrovascular disease. On examination, she had a left-sided hemiplegia but no other abnormal findings. Routine blood tests, electrocardiography, and the chest radiography were all normal. Computed tomography of the brain showed an infarct in the right basal ganglia but also other older infarcts in the left thalamus and left internal capsule. Thrombophilia and autoimmune screens were negative. Transesophageal echocardiography (Figure 1) showed a mobile, pedunculated tumor in the left atrial appendage. There was no spontaneous echo contrast, and the left atrial appendage had a normal Doppler flow pattern. The findings were suggestive of an atypical myxoma. The tumor was excised, and histopathologic examination showed it to be a papillary fibroelastoma.The patient had an uncomplicated postoperative recovery.
The left atrial appendage (LAA) is an unusual site for tumor formation, with only isolated case reports of myxomas or malignant tumors of the LAA.2,3 To our knowledge, this is the first case of a papillary fibroelastoma of the LAA to be reported.A mass localized to the LAA usually represents thrombus, especially if associated with atrial fibrillation, spontaneous echo contrast, or abnormal LAA emptying. In the reported case, none of these features were present and the mass was pedunculated, all of which suggested that it may be an atypical myxoma. It was therefore surprising when histology revealed a papillary fibroelastoma (PF). Papillary fibroelastomas are benign, avascular tumors derived from normal components of the endocardium. They are usually pedunculated, with multiple papillary fronds, giving the appearance of a sea anemone.1 Histologically, they consist of a central core of loose connective tissue covered by endothelium.They constitute 16% of all primary cardiac tumors but are the most common type of primary valve tumor.4,5 They tend to arise in areas of endocardial damage, for example, sites of valve sclerosis, rheumatic heart disease, or cardiac surgery.1 Nonvalvular papillary fibroelastomas have been reported on the septum or left ventricular outflow tract and right atrium but never before in the left atrial appendage.1 Typical echocardiographic features of PF are a small, pedunculated, mobile mass (diameter up to 20 mm) with a stippled edge, attached by a small stalk located at the midportion or body of the valve. Because of its rarity, the clinical characteristics of PFs are poorly understood. Other case reports and pathology series suggest that these friable tumors
From the Department of Echocardiography, Northwick Park Hospital, Middlesex, United Kingdom. Reprint requests: Dr R. Senior, MD, Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ, United Kingdom (E-mail: [email protected]). Copyright © 2001 by the American Society of Echocardiography. 0894-7317/2001/$35.00 + 0 27/4/114010 doi:10.1067/mje.2001.114010
838
Journal of the American Society of Echocardiography Volume 14 Number 8
Sidhu, Harries, Senior 839
have a potential to embolize either with tumor fragments or thrombus formed around the tumor.6 Although most left-sided PFs probably remain asymptomatic, they have a potential to cause serious complications such as embolic events or coronary artery obstruction.1,5,7 Hence the consensus view is to remove all PFs except for asymptomatic, right-sided tumors, which rarely go on to cause clinical complications.7 This case, as well as being unique, illustrates how the treatment of most benign cardiac tumors is governed by their location and symptomology rather than their histologic type. Figure 1 Transesophageal echocardiogram: 1.5-cm-diameter, pedunculated, mobile tumor (arrows) in left atrial appendage. LA, Left atrium; LV, left ventricle.
REFERENCES 1. Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathological correlation. J Am Coll Cardiol 1997;30:784-90. 2. Feinglass NG, Reeder GS, Finck SJ, Shine TS, Maniu CV. Myxoma of the left atrial appendage mimicking thrombus during aortic valve replacement. J Am Soc Echocardiogr 1998;11:677-9. 3. Boey S, Fouda-Omgba F, Mirode A, Tribouilloy, Quere JP, Lesbre JP. Malignant cardiac lymphoma: diagnosis by echocardiography. Arch Mal Coeur Vaiss 1998;90:1655-61. 4. Allard MF, Taylor GP, Wilson JE, McManus BM. Primary cardiac tumors. In Goldhaber SZ, Braunwald E, editors.
Cardiopulmonary diseases and cardiac tumors. Atlas of heart diseases. Vol 3. Philadelphia: Current Medicine 1995;15:115,22. 5. Edwards FH, Hale D, Cohen A, Thompson, Pezella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-31. 6. Burke A, Virmani R. Tumors of the heart and great vessels. In: Atlas of tumor pathology. Third series. Washington DC: American Forces Institute of Pathology; 1996:47-54. 7. Yee HC, Nwosu JE, Lii AD, Velasco M, Millman A. Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 1997;80:811-4.
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Cardiac papillary fibroelastomas are benign tumors that usually arise from the valvular endocardium. They are clinically important because of their propensity to embolize. We describe, to the best of our knowledge, the first reported case of a papillary fi-
broelastoma arising from the left atrial appendage, giving rise to multiple cerebral embolic events. The tumor was excised surgically, with no further embolic events. (J Am Soc Echocardiogr 2001;14:838-9.)
CASE REPORT
DISCUSSION
Papillary fibroelastomas are rare, benign tumors that arise almost exclusively from valvular endocardium.They have a well-known potential for causing systemic embolization.1 We describe, to the best of our knowledge, the first reported case of a papillary fibroelastoma of the left atrial appendage.The tumor was the cause of recurrent cerebral emboli and was successfully excised. A 59-year-old, right-handed woman had sudden weakness of the left arm and left leg. She had no history of cardiac symptoms or any risk factors for cerebrovascular disease. On examination, she had a left-sided hemiplegia but no other abnormal findings. Routine blood tests, electrocardiography, and the chest radiography were all normal. Computed tomography of the brain showed an infarct in the right basal ganglia but also other older infarcts in the left thalamus and left internal capsule. Thrombophilia and autoimmune screens were negative. Transesophageal echocardiography (Figure 1) showed a mobile, pedunculated tumor in the left atrial appendage. There was no spontaneous echo contrast, and the left atrial appendage had a normal Doppler flow pattern. The findings were suggestive of an atypical myxoma. The tumor was excised, and histopathologic examination showed it to be a papillary fibroelastoma.The patient had an uncomplicated postoperative recovery.
The left atrial appendage (LAA) is an unusual site for tumor formation, with only isolated case reports of myxomas or malignant tumors of the LAA.2,3 To our knowledge, this is the first case of a papillary fibroelastoma of the LAA to be reported.A mass localized to the LAA usually represents thrombus, especially if associated with atrial fibrillation, spontaneous echo contrast, or abnormal LAA emptying. In the reported case, none of these features were present and the mass was pedunculated, all of which suggested that it may be an atypical myxoma. It was therefore surprising when histology revealed a papillary fibroelastoma (PF). Papillary fibroelastomas are benign, avascular tumors derived from normal components of the endocardium. They are usually pedunculated, with multiple papillary fronds, giving the appearance of a sea anemone.1 Histologically, they consist of a central core of loose connective tissue covered by endothelium.They constitute 16% of all primary cardiac tumors but are the most common type of primary valve tumor.4,5 They tend to arise in areas of endocardial damage, for example, sites of valve sclerosis, rheumatic heart disease, or cardiac surgery.1 Nonvalvular papillary fibroelastomas have been reported on the septum or left ventricular outflow tract and right atrium but never before in the left atrial appendage.1 Typical echocardiographic features of PF are a small, pedunculated, mobile mass (diameter up to 20 mm) with a stippled edge, attached by a small stalk located at the midportion or body of the valve. Because of its rarity, the clinical characteristics of PFs are poorly understood. Other case reports and pathology series suggest that these friable tumors
From the Department of Echocardiography, Northwick Park Hospital, Middlesex, United Kingdom. Reprint requests: Dr R. Senior, MD, Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ, United Kingdom (E-mail: [email protected]). Copyright © 2001 by the American Society of Echocardiography. 0894-7317/2001/$35.00 + 0 27/4/114010 doi:10.1067/mje.2001.114010
838
Journal of the American Society of Echocardiography Volume 14 Number 8
Sidhu, Harries, Senior 839
have a potential to embolize either with tumor fragments or thrombus formed around the tumor.6 Although most left-sided PFs probably remain asymptomatic, they have a potential to cause serious complications such as embolic events or coronary artery obstruction.1,5,7 Hence the consensus view is to remove all PFs except for asymptomatic, right-sided tumors, which rarely go on to cause clinical complications.7 This case, as well as being unique, illustrates how the treatment of most benign cardiac tumors is governed by their location and symptomology rather than their histologic type. Figure 1 Transesophageal echocardiogram: 1.5-cm-diameter, pedunculated, mobile tumor (arrows) in left atrial appendage. LA, Left atrium; LV, left ventricle.
REFERENCES 1. Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathological correlation. J Am Coll Cardiol 1997;30:784-90. 2. Feinglass NG, Reeder GS, Finck SJ, Shine TS, Maniu CV. Myxoma of the left atrial appendage mimicking thrombus during aortic valve replacement. J Am Soc Echocardiogr 1998;11:677-9. 3. Boey S, Fouda-Omgba F, Mirode A, Tribouilloy, Quere JP, Lesbre JP. Malignant cardiac lymphoma: diagnosis by echocardiography. Arch Mal Coeur Vaiss 1998;90:1655-61. 4. Allard MF, Taylor GP, Wilson JE, McManus BM. Primary cardiac tumors. In Goldhaber SZ, Braunwald E, editors.
Cardiopulmonary diseases and cardiac tumors. Atlas of heart diseases. Vol 3. Philadelphia: Current Medicine 1995;15:115,22. 5. Edwards FH, Hale D, Cohen A, Thompson, Pezella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-31. 6. Burke A, Virmani R. Tumors of the heart and great vessels. In: Atlas of tumor pathology. Third series. Washington DC: American Forces Institute of Pathology; 1996:47-54. 7. Yee HC, Nwosu JE, Lii AD, Velasco M, Millman A. Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 1997;80:811-4.
Receive tables of contents by e-mail To receive the tables of contents by e-mail, sign up through our Web site at http://www.mosby.com/echo Choose E-mail Notification. Simply type your e-mail address in the box and click the Subscribe button. Alternatively, you may send an e-mail message to [email protected]. Leave the subject line blank, and type the following as the body of your message: subscribe echo_toc You will receive an e-mail message to confirm that you have been added to the mailing list. Note that table of contents e-mail messages will be sent when a new issue is posted to the Web site.