Ann Thorac Surg 1996;62:1519-1521
© 1996 The Society of Thoracic Surgeons
Case Report
Cardiac Papillary Fibroelastomas: Rationale for Excision
Kenji Minatoya, MD,
Hitoshi Okabayashi, MD, PhD,
Tadaaki Yokota, MD, PhD,
Eddie L. Hoover, MD
Divisions of Cardiovascular Surgery and Pathology, Kokura Memorial Hospital, Kitakyusyu, Japan
Accepted for publication May 24, 1996.
 |
Abstract
|
|---|
We experienced a case of papillary fibroelastoma of the left ventricular outflow tract in a patient with severe valvular heart disease that was detected only by transesophageal echocardiography. Preoperative detection of this lesion altered the surgical procedure to include resection of the mass through the aortic valve annulus along with repair/replacement of the valves. The literature documents sufficient morbidity/mortality to support excision of these lesions regardless of symptoms or location.
|
Introduction
|
|---|
Primary cardiac tumors continue to be rare clinical entities with a frequency of less than 0.3% from collected clinical and postmortem series. Papillary fibroelastoma, commonly referred to as the "third primary cardiac tumor," is still extremely uncommon and was formerly found primarily at autopsy. This lesion most frequently arises from valvular endocardium and is usually small and asymptomatic [1]. Reports of surgical excision are becoming more frequent because of the advent of ultrasound cardiography and transesophageal echocardiography (TEE). We report the case of a patient with a papillary fibroelastoma of the left ventricular outflow tract that was discovered on TEE during evaluation for valvular heart disease. The role of TEE and surgical intervention constitute the basis of this report.
A 53-year-old woman was admitted to Kokura Memorial Hospital for evaluation of recent attacks of dyspnea on exertion. She was known to have chronic valvular diseases and a history of childhood rheumatic fever. Electrocardiogram showed atrial fibrillation. After transthoracic echocardiography and cardiac catheterization she was finally diagnosed as having severe mitral stenosis, mild mitral regurgitation, moderate tricuspid regurgitation, and mild aortic regurgitation. She was scheduled for elective mitral valve replacement and tricuspid annuloplasty, but TEE detected a small mass fluttering along the left ventricular outflow tract that was not noticed with routine transthoracic echocardiography (Fig 1
). It was thought to be a vegetation because its location corresponded to the impact area of the regurgitant jet from the aortic valve. Although she was afebrile and had no other evidence of infection, she underwent semiemergency operation because of this presumptive diagnosis.
View larger version (127K):
[in this window]
[in a new window]
|
Fig 1. . Transesophageal echocardiogram demonstrating a small, well-circumscribed density along the outflow tract of the left ventricle (arrows).
|
|
The lesion was identified through the aortic valve annulus and was attached to the anterior wall of the left ventricular outflow tract. The size of the tumor resected was 7 x 2 mm. It had a smooth, rubbery surface and its color was yellow-white (Fig 2). This was not the typical "hairy" form of fibroelastoma. After aortic valvuloplasty, mitral valve replacement, and tricuspid annuloplasty, the patient was weaned from bypass without difficulty, and the postoperative course was uneventful.
View larger version (147K):
[in this window]
[in a new window]
|
Fig 2. . Photomicrograph demonstrating the gross appearance of the resected lesion (arrows), which is attached to a small segment of endocardium.
|
|
Histologic examination of the tumor showed a rim of elastic fiber and a myxoid matrix with collagen fibers (Fig 3). The outer layer consisted of regular endothelial cells; therefore, we concluded that it was a papillary fibroelastoma.
View larger version (107K):
[in this window]
[in a new window]
|
Fig 3. . Light microscopy of the lesion demonstrating a myxoid matrix, collagen fibers, and a border of elastic fibers. (Hematoxylin and eosin; x100 before 54% reduction.)
|
|
|
Comment
|
|---|
Papillary fibroelastoma is the third most frequent benign tumor of the heart after myxoma and lipoma. Papillary fibroelastomas are derived from the endocardium and, as such, consist of the normal components of the endocardium including fibrous tissue, elastic fibers, and smooth muscle cells. They have a valvular predilection and the vast majority are incidental findings at autopsy or on surgically excised valves and are not associated with cardiac symptoms [1]. It was initially thought that this tumor was not clinically significant, but there are several recent reports of serious symptoms and complications that could be attributed to tumor thromboembolism or myocardial ischemia [2, 3].
Transesophageal echocardiography is proving to be superior to transthoracic echocardiography in cardiac imaging. Narang and colleagues [4] reported a case of an aortic valve fibroelastoma discovered intraoperatively by TEE that led to a change in the planned operation from coronary artery bypass grafting to include an aortotomy and excision of the tumor. In our patient we were unaware of the existence of the tumor until TEE was done, and we had not planned to perform an aortotomy because aortic regurgitation was estimated not to need surgical repair. In addition to being a better diagnostic test, TEE is also an excellent operative guide.
Papillary fibroelastoma was first described as gigantic Lambl's excrescences [5]. It has recently been suggested that the structure of the lesion consists of fibrin deposition over minor endothelial damage with subsequent organization of a mural thrombus. In typical cases papillary fibroelastoma resembles a sea anemone with multiple papillary fronds attached to the endocardium by a short pedicle [1]. In our case the shape of the tumor was identical to the chordae tendineae of the mitral valve, and there was no frond formation macroscopically.
Lee and associates [6] described an atypical presentation of papillary fibroelastoma mimicking multiple vegetations in suspected subacute bacterial endocarditis. In our case, although the patient had no fever and no other evidence of infection, we could not rule out a vegetation because its site coincided with the region of the regurgitant jet. Therefore, she underwent semiemergency operation.
When this lesion is found in a patient with symptoms, then operation is clearly indicated. However, when it is an incidental finding, one might be tempted to make a case for observation. In 1991 Neerukonda and colleagues [7] demonstrated that papillary fibroelastoma can be fatal even in the right side of the heart. Because left-sided lesions have been shown to cause cardiac and cerebral symptoms, we think that surgical excision is indicated in all patients regardless of symptoms or site of origin. Transesophageal echocardiography appears to be superior to transthoracic echocardiography in diagnosing these tumors and should be the preferred diagnostic procedure in the preoperative evaluation of patients with valvular heart disease or suspected cardiac tumors.
|
Footnotes
|
|---|
Address reprint requests to Dr Minatoya, Division of Cardiovascular Surgery, Kokura Memorial Hospital, 1-1 Kifunecho, Kokurakita-ku, Kitakyusyu, Fukuoka 802, Japan.
|
References
|
|---|
- McAllister HA Jr, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology. Second series, fascicle 15. Washington, DC: Armed Forces Institute of Pathology, 1978.
- Kasarskis EJ, O'Connor W, Earle G. Embolic stroke from cardiac papillary fibroelastoma. Stroke 1988;19:1171–3.[Abstract/Free Full Text]
- Eckstein FS, Schafers HJ, Grote J, Mugge A, Borst HG. Papillary fibroelastoma of the aortic valve presenting with myocardial infraction. Ann Thorac Surg 1995;60:206–8.[Abstract/Free Full Text]
- Narang J, Neustein S, Israel D. The role of transesophageal echocardiography in the diagnosis and excision of a tumor of the aortic valve. J Cardiothorac Vasc Anesth 1992;6:68–9.[Medline]
- Boone S, Campagna M, Walley VM. Lambl's excrescences and papillary fibroelastomas: are they different? Can J Cardiol 1992;8:372–6.[Medline]
- Lee KS, Topol EJ, Stewart WJ. Atypical presentation of papillary fibroelastoma mimicking multiple vegetations in suspected subacute bacterial endocarditis. Am Heart J 1993;125:1443–5.[Medline]
- Neerukonda SK, Jantz RD, Vijay NK, Narrod JA, Scoonmaker FW. Pulmonary embolization of papillary fibroelastoma arising from the tricuspid valve. Tex Heart Inst J 1991;18:132–5.[Medline]
This article has been cited by other articles:
|
|
|
|
 
S. Mohammadi, A. Martineau, P. Voisine, and F. Dagenais
Left Atrial Papillary Fibroelastoma: A Rare Cause of Multiple Cerebral Emboli
Ann. Thorac. Surg.,
October 1, 2007;
84(4):
1396 - 1397.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. L. Grebenc, M. L. Rosado de Christenson, A. P. Burke, C. E. Green, and J. R. Galvin
Primary Cardiac and Pericardial Neoplasms: Radiologic-Pathologic Correlation
RadioGraphics,
July 1, 2000;
20(4):
1073 - 1103.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. A. Howard, G. S. Aldea, O. M. Shapira, J. M. Kasznica, and R. Davidoff
Papillary fibroelastoma: increasing recognition of a surgical disease
Ann. Thorac. Surg.,
November 1, 1999;
68(5):
1881 - 1885.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
