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Eur J Echocardiography (2006) 7, 253e256 Incidental finding of a large pulmonary valve fibroelastoma: A case report Marcos Daccarett a,*, Peter Burke b, Souheil Saba a a Division of Cardiology, Providence Hospital and Medical Center, 16001 W. 9 Mile Road, Southfield, MI 48075, USA b Department of Internal Medicine, Providence Hospital and Medical Center, 16001 W. 9 Mile Road, Southfield, MI 48075, USA KEYWORDS Large fibroelastoma; Pulmonary valve; Transesophageal echocardiogram; TEE; Asymptomatic incidental Abstract Papillary fibroelastoma (PFE) is an uncommon primary neoplasm of cardiac origin. They are solitary neoplasms that historically were an incidental finding at the time of autopsy. With the advent of two-dimensional echocardiography, symptomatic cases have been reported in current literature, thus causing a paradigm shift in the management of these tumors. Although the majority of PFE are benign, because of their potential risk for complication related to embolic and obstructive phenomena, they are now considered hazardous and require tumor excision even in asymptomatic patients. We report a case of an asymptomatic incidental large papillary fibroelastoma within the right ventricular outflow tract. ª 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved. Background Primary intracardiac tumors are extremely rare with an overall incidence of !0.3%.1 Papillary fibroelastomas comprise only 8% of all primary cardiac tumors, of which the vast majority are benign.2,3 Our understanding of papillary fibroelas- * Corresponding author. Tel.: C1 248 544 0515. E-mail addresses: [email protected] (M. Daccarett), [email protected] (P. Burke), [email protected] (S. Saba). tomas (PFE) has changed from an innocuous postmortem finding to a potentially life threatening pathology. Historically, small right-sided PFEs discovered on routine echocardiogram in asymptomatic patients were observed clinically.4 Twodimensional echocardiography on symptomatic patients, however, has demonstrated that these tumors have a tendency for embolization and obstruction resulting in cerebral vascular accidents, pulmonary embolism and myocardial infarction.5 It is now suggested that all PFE, regardless of size and location, should be resected to avoid embolic or occlusive-related complications.1,6,7 To 1525-2167/$32 ª 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.euje.2005.04.008 Downloaded from by guest on October 27, 2016 Received 10 March 2005; accepted 27 April 2005 Available online 31 May 2005 254 our knowledge, only four cases of pulmonary valve PFE have been reported in previous literature. Our case demonstrates a large right-sided PFE in an asymptomatic man that historically could have been observed without intervention, but due to its potential for life threatening complications surgical intervention was carried out with excellent outcome. M. Daccarett et al. elastin at the core of each frond, coated with collagen and lined by flat endocardial cellse confirming the diagnosis of papillary fibroelastoma (Fig. 3). The patient had an uncomplicated postoperative course and one and half months follow up revealed no complications. Discussion Case report Figure 1 Transesophageal echocardiogram short axis view of the base of the heart obtained with multiplane transducer at 40 demonstrating a 1.4 by 1.5 cm mass on the ventricular surface of the pulmonic valve (arrow). LA Z left atrium, RVOT Z right ventricular outflow tract, AoV Z aortic valve, PV Z pulmonic valve. Downloaded from by guest on October 27, 2016 The patient, an asymptomatic 52-year-old AfricanAmerican male with known hypertension and dyslipidemia, was found to have a large mass in the right ventricular outflow tract on routine echocardiogram. Transesophageal echocardiogram performed for further evaluation, confirmed a mobile, 1.5 cm by 1.4 cm mass attached to the ventricular aspect of the pulmonic valve above the right ventricular outflow tract as seen in long (Fig. 1) and short axes (Fig. 2) of the valve. A coronary angiogram demonstrated normal coronary anatomy without significant disease. Surgical resection was recommended due to the tumor’s large size and mobility, resulting in high risk for embolization. A transverse arteriotomy of the main pulmonary artery was performed revealing a 1.4 ! 1.0 ! 1.3 cm friable, gelatinous mass fixed onto the ventricular aspect of the pulmonic valve. Gross and microscopic histopathology revealed multiple frond-like structures comprising dense PFE represent 8% of all primary cardiac tumors. They are the third most common cardiac primary tumor after myxomas (30%) and lipomas (10%), but are the most common valvular tumor as up to 90% occur on the valvular endocardium.2 PFEs have a predilection for the left side of the heart. The review of current literature demonstrates that symptomatic PFEs are more likely to be located on the left side of the heart.1,2,8 Similar investigations confirm that the most commonly affected valvular site is the aortic valve, followed by the mitral and tricuspid valves, respectively. The pulmonic valve is rarely involved.1,2,8 PFEs are usually small tumors, and it has been reported by Sun et al.4 that 99% of all PFEs were 20 mm or less. The mean age of diagnosis was approximately 60 years, although they have been reported in children and teenagers.9e11 The pathophysiology of these tumors remains unknown. Several possible theories have been reported including previous endothelial damage followed by thrombosis and organization, iatrogenic Large pulmonary valve fibroelastoma: A case report 255 Figure 2 Transesophageal echocardiogram short axis view of the pulmonic valve obtained with multiplane transducer at 0 demonstrating a spherical mass attached to the pulmonic valve (arrow). LA Z left atrium, Ao Z aorta, PV Z pulmonic valve. the brain, heart and lung resulting in stroke, myocardial infarction or pulmonary embolus. Therefore, its location as well as size and mobility determine potential risks and complications. Even though the majority of cases are asymptomatic because of their potential for life threatening complications, current literature suggests that even small PFEs should be excised.1,6,7 Figure 3 Histology. Surgical specimen demonstrating multiple papillary fronds with a central core of dense fibroelastic tissue (arrow), and loose connective tissue with mucopolysaccharides (hematoxylineeosin staining). Downloaded from by guest on October 27, 2016 factors and infectious processes related to cytomegalovirus.12 Cytomegalovirus has been recovered from PFEs suggesting a viral induction of the tumor. PFEs have a tendency to embolize due to their soft and friable anatomy, often associated with adherent thrombus.13,14 The common presenting symptoms are related to embolic phenomena to 256 Surgery is strongly indicated for patients with embolic events, occlusive-related symptoms due to left or right ventricular outflow tract obstruction, and as in our case, those with highly mobile or large tumors.15e18 The aforementioned case demonstrates a rare neoplasm with an unusual size and location that underwent successful resection avoiding potential catastrophic embolic consequences. M. Daccarett et al. 8. 9. 10. 11. References 13. 14. 15. 16. 17. 18. Downloaded from by guest on October 27, 2016 1. Schoondyke J, Buress J, Shabaneh B, Giorgadze T, Fahrig S, Whitaker J. Papillary fibroelastoma involving the left ventricular wall. Rev Cardiovasc Med 2003;4:184e7. 2. Sierig G, Vondrys D, Daehnert I. Papillary fibroelastoma of the left atrium in a 3-year-old boy. Images Paediatr Cardiol 2003;17:5e9. 3. Remadi J, Degandt A, Rakotoarivello Z. Cardiac papillary fibroelastoma of the mitral valve chordae. Heart 2004;90:1397. 4. Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687e93. 5. Cohn L, Pins M. Case 16-1997 e a 42-year-old woman with a pulmonic-valve mass. N Engl J Med 1997;336:1512e6. 6. de Virgilio C, Dubrow TJ, Robertson JM, Siegel S, Ginzton L, Nussmeier M, et al. Detection of multiple cardiac papillary fibroelastomas using transesophageal echocardiography. Ann Thorac Surg 1989;48:119e21. 7. LiMandri G, Homma S, Di Tullio MR, Hodges D, Arora R, Marboe C, et al. Detection of multiple papillary fibroelastomas 12. of the tricuspid valve by transesophageal echocardiography. J Am Soc Echocardiogr 1994;7:315e7. Darvishian F, Farmer P. Papillary fibroelastoma of the heart: report of two cases and review of the literature. Ann Clin Lab Sci 2001;31:291e6. Deodhar AP, Tometzki AJP, Hudson IN, Mankad PS. Aortic valve tumor causing acute myocardial infarction in a child. Ann Thorac Surg 1997;64:1482e4. de Menezes IC, Fragata J, Martins FM. Papillary fibroelastoma of the mitral valve in a 3-year-old child: case report. Pediatr Cardiol 1996;17:194e5. Raeburn C. Papillary fibroelastic hamartomas of the heart valves. J Path Bacteriol 1953;65:371e5. Grandmougin D, Fayad G, Moukassa D, Decoene C, Abolmaali K, Bodart JC, et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis 2000;9:832e41. Tanaka H, Narisawa T, Mori T, Masuda Y, Kishi D. Double primary left ventricular and aortic valve papillary fibroelastoma. Circ J 2004;68(5):504e6. Burke A, Virmani R. Tumors of the heart and great vessels. In: Atlas of tumor pathology. Third series, vol. 16. Washington, DC: Armed Forces Institute of Pathology; 1996. p. 47e54. Howard RA, Aldea GS, Shapira OM, Kasznica JM, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881e5. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelastoma and stroke. Echocardiographic diagnosis and guide to excision. Am J Med 1986;80:129e32. Marvasti MA, Obeid AI, Cohen PS, Giambartolomei A, Parker FB. Successful removal of papillary endocardial fibroma. Thorac Cardiovasc Surg 1983;31:254e5. Shahian DM. Papillary fibroelastomas. 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