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Papillary Fibroelastoma Arising from the Left Atrial Free Wall
Papillary Fibroelastoma Arising from the Left Atrial Free Wall Osamudiamen Idahosa, MD, Vamshi Mallavarapu, MD, and Emanuel Kostacos, MD, Abington, Pennsylvania
Primary cardiac tumors are rare. Papillary fibroelastomas represent the second or third most common benign cardiac tumor and the most common one associated with heart valves. Nonvalvular endocar-
dial location of this tumor is extremely rare. We present a case of a papillary fibroelastoma arising from the left atrial (LA) free wall. (J Am Soc Echocardiogr 2007;20:1010.e1-e2.)
CASE REPORT
DISCUSSION
A 79-year-old man with a history of lower extremity deep venous thrombosis and transient ischemic attack 10 years prior presented with transient forearm weakness. He denied any cardiac symptoms and had no history of atrial fibrillation or structural heart disease. Physical examination was unremarkable. Transthoracic echocardiogram results obtained during the workup were reported as normal. He was seen by a neurologist, who referred him for a transesophageal echocardiogram for further evaluation. Transesophageal echocardiography showed a 1.5- ⫻ 1.5-cm mobile mass attached to the posterolateral LA free wall, between the ostium of the left superior pulmonary vein and the LA appendage. The mass appeared pedunculated with mobile fronds. His left ventricular systolic function was normal, there was mild thickening of the mitral and aortic valve leaflets and trace mitral regurgitation, but no evidence of aortic stenosis or regurgitation. He underwent resection of the mass. Gross pathology described a soft, friable, gelatinous tan mass with fibrous lining confined to the endocardial surface. Microscopic sections showed fibrous papilla lined by a single layer of bland cells with focal elastic fibers and areas of myxoid change. The morphologic appearance was most consistent with a papillary fibroelastoma.
From Internal Medicine (O.I., V.M.) and Cardiology Division (E.K.), Abington Memorial Hospital. Reprint requests: Osamudiamen Idahosa, MD, Department of Internal Medicine and Cardiology, Abington Memorial Hospital, 1200 Old York Rd, Abington, PA 19001 (E-mail: [email protected]). 0894-7317/$32.00 Copyright 2007 by the American Society of Echocardiography. doi:10.1016/j.echo.2007.01.027
Papillary fibroelastomas are benign tumors comprising less than 10% of all primary cardiac tumors.1-7 Approximately 80% are associated with valvular endothelium.4 They are most often found on the aortic and mitral valves, less commonly on tricuspid and pulmonary valves, and rarely along the atrial or ventricular walls.5 To date, there is only one other report in the literature of a papillary fibroelastoma arising from the posterolateral surface of the LA free wall.2 It is a very unusual site for this tumor, which can present as solitary or multiple lesions. Patients are often asymptomatic, detected incidentally at autopsy, during surgery, or on routine echocardiography.3 These tumors have been associated with stroke, angina, myocardial infarction, ventricular outflow obstruction, syncope, and embolic phenomena. Transesophageal echocardiography is the diagnostic procedure of choice. Echocardiographic features of papillary fibroelastomas include: (1) small lesions, typically less than 1 cm in diameter but may be as large as 3 to 4 cm; (2) highly mobile with a pedicle or stalk attachment to the valve or endocardium; and (3) frondlike appearance.3,8 Grossly, papillary fibroelastomas appear as small avascular tumors with multiple papillary fronds arising from a central core or stalk “resembling a sea anemone.”3,7 Histologically, they consist of a core of fibrous tissue, elastic fibers, and collagen fibers with an outer layer of regular endothelial cells.3,7 Surgical excision is recommended because of the risk of embolic events.3,4,5,7 Anticoagulation is generally not considered adequate therapy. Recurrence rates after surgical excision have not been reported, but follow-up is recommended.6 Conclusion In summary, this is the rare case of a patient presenting with a transient ischemic attack caused by a papillary fibroelastoma arising from the LA free wall.
1010.e1
1010.e2 Idahosa, Mallavarapu, Kostacos
Journal of the American Society of Echocardiography August 2007
Figure Transesophageal echocardiogram showing a short axis view at athe level of the aortic valve with a pedunculated mass with mobile fronds lying between the left atrial appendage and left superior pulmonary vein. REFERENCES 1. Davoli G, Muzzi L, Lucchese G, Uricchi N, et al. Double papillary fibroelastoma of the aortic valve. Tex Heart Inst J 2004;31:448-9. 2. Wolber T, Facchini M, Huerlimann S, Rickli H. Papillary fibroelastoma of the left atrial free wall. Circulation 2001;104:e87-8. 3. Gowda MR, Khan IA, Mehta NJ. Cardiac papillary fibroelastoma originating from pulmonary vein. Angiology 2002;53:745-8. 4. Howard RA, Aldea GS, Shapira OM, Kasznica JM, et al. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881-5.
5. Eslami-Varzaneh F, Brun EA, Sears-Ragan P. An unusual case of multiple papillary fibroelastoma: review of literature. Cardiovasc Pathol 2003;12:170-3. 6. Yeo KK, Fukuyama O. Multiple cardiac papillary fibroelastoma and transient left ventricular apical ballooning syndrome in an elderly woman: case report. J Heart Valve Dis 2005;14:137-9. 7. Butany J, Nair V, Ahluwaha MS, El Demellawy D, et al. Papillary fibroelastoma of the interatrial septum. J Card Surg 2004;19:349-53. 8. Daveron E, Jain Neeraj, et al. Papillary fibroelastoma and lambl’s excrescences: echocardiographic diagnosis and differential diagnosis. Echocardiography 2005;22:461-3.
Primary cardiac tumors are rare. Papillary fibroelastomas represent the second or third most common benign cardiac tumor and the most common one associated with heart valves. Nonvalvular endocar-
dial location of this tumor is extremely rare. We present a case of a papillary fibroelastoma arising from the left atrial (LA) free wall. (J Am Soc Echocardiogr 2007;20:1010.e1-e2.)
CASE REPORT
DISCUSSION
A 79-year-old man with a history of lower extremity deep venous thrombosis and transient ischemic attack 10 years prior presented with transient forearm weakness. He denied any cardiac symptoms and had no history of atrial fibrillation or structural heart disease. Physical examination was unremarkable. Transthoracic echocardiogram results obtained during the workup were reported as normal. He was seen by a neurologist, who referred him for a transesophageal echocardiogram for further evaluation. Transesophageal echocardiography showed a 1.5- ⫻ 1.5-cm mobile mass attached to the posterolateral LA free wall, between the ostium of the left superior pulmonary vein and the LA appendage. The mass appeared pedunculated with mobile fronds. His left ventricular systolic function was normal, there was mild thickening of the mitral and aortic valve leaflets and trace mitral regurgitation, but no evidence of aortic stenosis or regurgitation. He underwent resection of the mass. Gross pathology described a soft, friable, gelatinous tan mass with fibrous lining confined to the endocardial surface. Microscopic sections showed fibrous papilla lined by a single layer of bland cells with focal elastic fibers and areas of myxoid change. The morphologic appearance was most consistent with a papillary fibroelastoma.
From Internal Medicine (O.I., V.M.) and Cardiology Division (E.K.), Abington Memorial Hospital. Reprint requests: Osamudiamen Idahosa, MD, Department of Internal Medicine and Cardiology, Abington Memorial Hospital, 1200 Old York Rd, Abington, PA 19001 (E-mail: [email protected]). 0894-7317/$32.00 Copyright 2007 by the American Society of Echocardiography. doi:10.1016/j.echo.2007.01.027
Papillary fibroelastomas are benign tumors comprising less than 10% of all primary cardiac tumors.1-7 Approximately 80% are associated with valvular endothelium.4 They are most often found on the aortic and mitral valves, less commonly on tricuspid and pulmonary valves, and rarely along the atrial or ventricular walls.5 To date, there is only one other report in the literature of a papillary fibroelastoma arising from the posterolateral surface of the LA free wall.2 It is a very unusual site for this tumor, which can present as solitary or multiple lesions. Patients are often asymptomatic, detected incidentally at autopsy, during surgery, or on routine echocardiography.3 These tumors have been associated with stroke, angina, myocardial infarction, ventricular outflow obstruction, syncope, and embolic phenomena. Transesophageal echocardiography is the diagnostic procedure of choice. Echocardiographic features of papillary fibroelastomas include: (1) small lesions, typically less than 1 cm in diameter but may be as large as 3 to 4 cm; (2) highly mobile with a pedicle or stalk attachment to the valve or endocardium; and (3) frondlike appearance.3,8 Grossly, papillary fibroelastomas appear as small avascular tumors with multiple papillary fronds arising from a central core or stalk “resembling a sea anemone.”3,7 Histologically, they consist of a core of fibrous tissue, elastic fibers, and collagen fibers with an outer layer of regular endothelial cells.3,7 Surgical excision is recommended because of the risk of embolic events.3,4,5,7 Anticoagulation is generally not considered adequate therapy. Recurrence rates after surgical excision have not been reported, but follow-up is recommended.6 Conclusion In summary, this is the rare case of a patient presenting with a transient ischemic attack caused by a papillary fibroelastoma arising from the LA free wall.
1010.e1
1010.e2 Idahosa, Mallavarapu, Kostacos
Journal of the American Society of Echocardiography August 2007
Figure Transesophageal echocardiogram showing a short axis view at athe level of the aortic valve with a pedunculated mass with mobile fronds lying between the left atrial appendage and left superior pulmonary vein. REFERENCES 1. Davoli G, Muzzi L, Lucchese G, Uricchi N, et al. Double papillary fibroelastoma of the aortic valve. Tex Heart Inst J 2004;31:448-9. 2. Wolber T, Facchini M, Huerlimann S, Rickli H. Papillary fibroelastoma of the left atrial free wall. Circulation 2001;104:e87-8. 3. Gowda MR, Khan IA, Mehta NJ. Cardiac papillary fibroelastoma originating from pulmonary vein. Angiology 2002;53:745-8. 4. Howard RA, Aldea GS, Shapira OM, Kasznica JM, et al. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881-5.
5. Eslami-Varzaneh F, Brun EA, Sears-Ragan P. An unusual case of multiple papillary fibroelastoma: review of literature. Cardiovasc Pathol 2003;12:170-3. 6. Yeo KK, Fukuyama O. Multiple cardiac papillary fibroelastoma and transient left ventricular apical ballooning syndrome in an elderly woman: case report. J Heart Valve Dis 2005;14:137-9. 7. Butany J, Nair V, Ahluwaha MS, El Demellawy D, et al. Papillary fibroelastoma of the interatrial septum. J Card Surg 2004;19:349-53. 8. Daveron E, Jain Neeraj, et al. Papillary fibroelastoma and lambl’s excrescences: echocardiographic diagnosis and differential diagnosis. Echocardiography 2005;22:461-3.