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Pathologic quiz case: a symptomatic right atrial mass. (Residents' Pages).

A 59-year-old woman with a history of hypertension presented with acute onset of diplopia, which rapidly progressed to right facial and upper extremity paresthesia and weakness. The patient denied chest pain, shortness of breath, palpitations, or nausea. All vital signs were within normal limits, and no precordial murmurs, friction rubs, or thrills were present. An electrocardiogram showed normal sinus rhythm. Computed tomographic scan of the head was negative, a magnetic resonance image showed only mild small vessel ischemic changes in the brain, and a bilateral carotid Doppler examination was normal. Transthoracic echocardiogram revealed a heart of normal size, an ejection fraction of 60%, and a 1.5-cm right atrial mass that did not obstruct the tricuspid vane. Tricuspid regurgitation was not present. The patient's symptoms resolved completely within 18 hours of presentation. Further workup via a transesophageal echocardiogram showed a 2-cm right atrial mass (Figure 1, small arrowhead), which was arising directly from the tricuspid valve (Figure 1, large arrowhead). The patient underwent an open-heart resection of this mass.

[FIGURE 1 OMITTED]

Gross examination of the specimen revealed multiple fragments of a tan-yellow papillary mass measuring 3.2 x 2.8 x 1.4 cm in aggregate. Flotation of the mass in saline was reminiscent of a "sea anemone" with frondlike projections. Microscopic examination delineated a papillary structure with avascular cores composed of a hyalinized and focally myxoid stroma with scattered stellate fibroblasts (Figure 2). The papillary structures were lined by a simple, flattened endothelial lining in continuity with the normal valvular endothelium. An elastin stain of the papillae demonstrated a central, loosely lamellated elastic tissue core with tapering rudimentary elastin fibers located toward the periphery (Figure 3).

[FIGURES 2-3 OMITTED]

What is your diagnosis?

Pathologic Diagnosis: Papillary Fibroelastoma

Papillary fibroelastomas (PFEs) are rare, benign cardiac tumors that comprise only 8% of all heart tumors. However, they are the third most common heart tumor after myxomas and lipomas, and they are the most frequent primary tumor of the heart valves. (1-4) In autopsy series, they are found on both the left and right side of the heart with almost equal frequency, and they are uncommonly (<20%) found without attachment to a cardiac valve. (3) Overall, the most common site is the aortic valve. Prior to modern imaging studies, PFEs were diagnosed exclusively at autopsy. The technological advancements of echocardiographic imaging, particularly transesophageal echocardiography, have made antemortem diagnosis possible. Grossly, these tumors are friable, white to yellow masses (often with adherent thrombus), which have been likened to sea anemones for their delicate frondlike papillary structures. Histologically, the papillae are characterized by a central, avascular, fibroelastotic stroma surrounded by acid mucopolysaccharide and covered by hyperplastic to focally attenuated endothelial cells.

The etiology of these tumors is currently unknown, although several theories have been postulated. These theories include postinflammatory rheumatic heart disease or possibly mechanical trauma as causes of an endothelial cell hyperplasia. Others have proposed that papillary fibroelastomas may represent a neoplastic or hamartomatous process. (5) Papillary fibroelastomas have been referred to by a variety of names, including cardiac papilloma, myxofibroma, papillary fibroma, and giant Lambl excrescences. Most reports agree that they are best termed papillary fibroelastoma on morphologic and histologic grounds, and are not simply giant Lamb] excrescences. (3,6)

The vast majority of these tumors are asymptomatic and are discovered incidentally during echocardiography or cardiac catheterization. Studies have shown that related myocardial infarction, cerebrovascular ischemia, stroke, and sudden death are possible. (2-4) Presumably, fragments of a thrombus, which often forms on the surface of the tumor, embolize to cause these symptoms. Additionally, aortic valve PFEs may act as a ball valve to obstruct the coronary ostia, causing intermittent cardiac ischemia and myocardial infarction. Most of the studies have linked left-sided PFEs to transient ischemic attacks and strokes, and right-sided tumors to pulmonary embolism. However, rare reports of syncopal episodes, congestive heart failure, and transient ischemic attacks due to a tricuspid papillary fibroelastoma have been reported. (1,6)

Treatment of asymptomatic cases ranges from no intervention to warfarin anticoagulation to surgical removal. Treatment of symptomatic PFEs consists of surgical removal of the tumor with native valve-sparing open-heart surgery. In this particular case, the tricuspid papillary fibroelastoma was thought to be the cause of this patient's transient ischemic attack, manifesting as right arm and facial paresthesia and weakness. The patient, therefore, underwent surgical resection with valvular sparing. The procedure was uneventful, and the patient has fully recovered without tricuspid valvular dysfunction or other sequelae.

References

(1.) Ganjoo AK, Johnson WD, Gordon RT, et al. Tricuspid papillary fibroelastoma causing syncopal episodes. J Thorac Cardiovasc Surg. 1996;112:551-552.

(2.) Klarich KW, Enriquez-Sarano M, Gura GM, et al. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coil Cardiol. 1997;30:784-790.

(3.) Howard RA, Aldea GS, Shapira OM, et al. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg. 1999;68:1881-1885.

(4.) Yee HC, Nwosu JE, Lii AD, et al. Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol. 1997;80: 811-814.

(5.) Heath D, Best V, Davis T. Papilliferous tumours of the heart valves. Br Heart J. 1961;23:20-24.

(6.) McAllistar HA Jr, Fenoglio JJ Jr. Tumors of the Cardiovascular System. Washington, DC: Armed Forces Institute of Pathology; 1978:1-3, 20-25. Atlas of Tumor Pathology; 2nd series, fascicle 15.

Accepted for publication April 16, 2001.

From the Department of Pathology, Beth Israel-Deaconess Medical Center, Boston, Mass.

Reprints: Robert A. Schwartz, MD, Department of Pathology, Beth Israel-Deaconess Medical Center, Boston, MA 02215 (e-mail: schwartzra@hotmail.com).
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Title Annotation:papillary fibroelastoma
Author:Schwartz, Robert A.; Kuten, Adam R.
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:1USA
Date:Dec 1, 2001
Words:923
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