Copyright © 2005, The European Society of Cardiology
Incidental finding of a large pulmonary valve fibroelastoma: A case report
aDivision of Cardiology, Providence Hospital and Medical Center, 16001 W. 9 Mile Road, Southfield, MI 48075, USA souheil.saba{at}providence-stjohnhealth.org
bDepartment of Internal Medicine, Providence Hospital and Medical Center, 16001 W. 9 Mile Road, Southfield, MI 48075, USA peter.burke{at}stjohn.org
Received 10 March 2005; accepted after revision 27 April 2005.
* Corresponding author. Tel.: +1 248 544 0515. marcos.daccarett{at}stjohn.org
 |
Abstract |
|---|
 Top Abstract BackgroundCase reportDiscussionReferences |
|---|
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.
Keywords: Large fibroelastoma; Pulmonary valve; Transesophageal echocardiogram; TEE; Asymptomatic incidental
|
Background |
|---|
|
TopAbstractBackgroundCase reportDiscussionReferences |
|---|
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 fibroelastomas (PFE) has changed from an innocuous post-mortem finding to a potentially life threatening pathology. Historically, small right-sided PFEs discovered on routine echocardiogram in asymptomatic patients were observed clinically.4 Two-dimensional 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 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.
|
Case report |
|---|
|
TopAbstractBackgroundCase reportDiscussionReferences |
|---|
The patient, an asymptomatic 52-year-old African-American 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, 1.5cm by 1.4cm 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 1.4x1.0x1.3cm friable, gelatinous mass fixed onto the ventricular aspect of the pulmonic valve. Gross and microscopic histopathology revealed multiple frond-like structures comprising dense elastin at the core of each frond, coated with collagen and lined by flat endocardial cells– 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 |
|---|
|
TopAbstractBackgroundCase reportDiscussionReferences |
|---|
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 20mm or less. The mean age of diagnosis was approximately 60 years, although they have been reported in children and teenagers.9–11
The pathophysiology of these tumors remains unknown. Several possible theories have been reported including previous endothelial damage followed by thrombosis and organization, iatrogenic 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 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 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.15–18
The aforementioned case demonstrates a rare neoplasm with an unusual size and location that underwent successful resection avoiding potential catastrophic embolic consequences.
|
References |
|---|
|
TopAbstractBackgroundCase reportDiscussionReferences |
|---|
- Schoondyke J., Buress J., Shabaneh B., Giorgadze T., Fahrig S., Whitaker J. Papillary fibroelastoma involving the left ventricular wall. Rev Cardiovasc Med (2003) 4:184–187.[Medline]
- Sierig G., Vondrys D., Daehnert I. Papillary fibroelastoma of the left atrium in a 3-year-old boy. Images Paediatr Cardiol (2003) 17:5–9.
- Remadi J., Degandt A., Rakotoarivello Z. Cardiac papillary fibroelastoma of the mitral valve chordae. Heart (2004) 90:1397.
[Free Full Text] - Sun J.P., Asher C.R., Yang X.S., Cheng G.G., Scalia G.M., Massed A.G., et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation (2001) 103:2687–2693.
[Abstract/Free Full Text] - Cohn L., Pins M. Case 16-1997 – a 42-year-old woman with a pulmonic-valve mass. N Engl J Med (1997) 336:1512–1516.
[Free Full Text] - de Virgilio C., Dubrow T.J., Robertson J.M., Siegel S., Ginzton L., Nussmeier M., et al. Detection of multiple cardiac papillary fibroelastomas using transesophageal echocardiography. Ann Thorac Surg (1989) 48:119–121.[Abstract]
- LiMandri G., Homma S., Di Tullio M.R., Hodges D., Arora R., Marboe C., et al. Detection of multiple papillary fibroelastomas of the tricuspid valve by transesophageal echocardiography. J Am Soc Echocardiogr (1994) 7:315–317.[Medline]
- Darvishian F., Farmer P. Papillary fibroelastoma of the heart: report of two cases and review of the literature. Ann Clin Lab Sci (2001) 31:291–296.
[Abstract/Free Full Text] - Deodhar A.P., Tometzki A.J.P., Hudson I.N., Mankad P.S. Aortic valve tumor causing acute myocardial infarction in a child. Ann Thorac Surg (1997) 64:1482–1484.
[Abstract/Free Full Text] - de Menezes I.C., Fragata J., Martins F.M. Papillary fibroelastoma of the mitral valve in a 3-year-old child: case report. Pediatr Cardiol (1996) 17:194–195.[Web of Science][Medline]
- Raeburn C. Papillary fibroelastic hamartomas of the heart valves. J Path Bacteriol (1953) 65:371–375.[CrossRef][Web of Science][Medline]
- Grandmougin D., Fayad G., Moukassa D., Decoene C., Abolmaali K., Bodart J.C., et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis (2000) 9:832–841.[Web of Science][Medline]
- Tanaka H., Narisawa T., Mori T., Masuda Y., Kishi D. Double primary left ventricular and aortic valve papillary fibroelastoma. Circ J (2004) 68(5):504–506.[CrossRef][Web of Science][Medline]
- Burke A., Virmani R. Tumors of the heart and great vessels. In: Atlas of tumor pathology. Third series (1996) vol. 16. Washington, DC: Armed Forces Institute of Pathology. p. 47–54.
- Howard R.A., Aldea G.S., Shapira O.M., Kasznica J.M., Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg (1999) 68:1881–1885.
[Abstract/Free Full Text] - Topol E.J., Biern R.O., Reitz B.A. Cardiac papillary fibroelastoma and stroke. Echocardiographic diagnosis and guide to excision. Am J Med (1986) 80:129–132.[CrossRef][Web of Science][Medline]
- Marvasti M.A., Obeid A.I., Cohen P.S., Giambartolomei A., Parker F.B. Successful removal of papillary endocardial fibroma. Thorac Cardiovasc Surg (1983) 31:254–255.[Web of Science][Medline]
- Shahian D.M. Papillary fibroelastomas. Semin Thorac Cardiovasc Surg (2000) 12:101–110.[Medline]
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  S. Biocic, S. Puksic, J. Vincelj, Z. Durasevic, Z. Sutlic, and S. Manojlovic Pulmonary valve papillary fibroelastoma diagnosed by echocardiography: a case report Eur J Echocardiogr, July 1, 2009; 10(5): 726 - 728. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||