mitral valve mass with echocardiographic ap- ... valve * cerebrovascular accident* transesophageal ... either obstruction or ball valve occlusion of a coronary.
CASE REPORTS PSEUDOPAPILLARY FIBROELASTOMA OF THE MITRAL VALVE Ernest Madu, MD, Jonathan Myles, MD, and Theodore D. Fraker, Jr, MD Memphis, Tennessee and Toledo, Ohio
Papillary fibroelastomas are well-recognized benign cardiac neoplasms. They are primarily asymptomatic, but occasionally are associated with neurologic and cardiac symptoms. Pseudopapillary fibroelastomas presenting with usual clinical and echocardiographic manifestations of papillary fibroelastoma but lacking characteristic histologic features have not been described previously. This article describes a 42-year-old, previously healthy female admitted with sudden hemiparesis and dysarthria. Symptoms completely resolved within 4 days. Extensive investigations revealed no etiology except for a pedunculated mitral valve mass with echocardiographic appearance suggestive of papillary fibroelastoma. Histologic staining, however, failed to reveal characteristic features of papillary fibroelastoma. (J Nat! Med Assoc. 1995;87:6870.) Key words * pseudopapillary fibroelastoma * mitral valve * cerebrovascular accident * transesophageal echocardiogram Noninfectious cardiac valvular lesions are usually primary cardiac neoplasms. Eighty percent of these tumors are benign with an autopsy incidence of 0.002% to 0.33%.' Papillary fibroelastomas rank third in order of prevalence of these benign primary cardiac neoplasms after myxomas and lipomas. They were first From the Department of Medicine, Division of Cardiovascular Diseases, University of Tennessee, the Department of Medicine and the Department of Pathology, the Medical College of Ohio, Toledo, Ohio. Requests for reprints should be addressed to Dr Ernest C. Madu, Division of Cardiovascular Diseases, The UT Medical Ctr, 951 Court Ave, Rm 353D, Memphis, TN 38163. 68
described in 1981 by Shub et a12 in an asymptomatic patient. Characteristically, these tumors adhere to the mitral valve leaflet via a pedicle, giving an echocardiographic appearance of a pedunculated tumor.3 These tumors consist of delicate finger-like projections composed of a central core of collagen and elastin and the characteristic papillary fronds of cardiac fibroelas-
toma.4 This article describes a case of "pseudo" mitral valve papillary fibroelastoma diagnosed preoperatively by transesophageal echocardiogram (TEE) in a patient in whom the characteristic pathologic features of papillary fronds on histological stain were absent.
CASE REPORT A previously healthy 42-year-old female developed sudden hemiparesis and dysarthria compatible with an embolic stroke. These symptoms resolved within 4 days, and computed tomography scans showed no defect. An echocardiogram showed an abnormal mitral valve with a possible mass, but was otherwise normal. No other source of emboli was discovered in a comprehensive work-up. A subsequent TEE revealed a pedunculated, finger-like mass on the atrial surface of the anterior mitral valve leaflet (Figures 1 and 2). She was treated with warfarin anticoagulation for 1 month, and a TEE was repeated. The mass lesion persisted and appeared to be somewhat larger. Surgical resection was recommended for a presumed papillary fibroelastoma of the mitral valve. At surgery, the mitral valve appeared normal except for a 3-cm long mass attached to the valve at a 1 cm2 base. A thin stalk connected the base to the tip of the mass. Histologic examination revealed organized thrombus with no evidence of papillary fronds. Special stains showed no evidence of elastin or collagen strands (Figures 3 and 4). JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 87, NO. 1
CASE REPORT
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Figure 3. Gross specimen of the resected mitral valve leaflet. The chordae tendinae are at the top of the photo. The arrow denotes the "growth" present on the atrial surface of the valve.
Figure 1. Transesophageal echocardiogram showing pseudopapillary fibroelastoma of the mitral valve attached to the anterior mitral valve leaflet via a stalk.
Figure 2. Transesophageal echocardiogram showing pseudopapillary fibroelastoma of the
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Figure 4. Hematoxylin and eosin stained section of lesion. Note the thrombus indicated by the arrow with the underlying valve parenchyma at the left. The entire lesion was composed of recent and organizing thrombus. There was no evidence of papillary fibroelastoma (magnification x 25).
mitral valve.
DISCUSSION Cardiac papillary fibroelastoma is a rare tumor, and until recently, it usually was discovered at autopsy. With the advent of 2-D echocardiography, however, a limited number have been detected antemortem. This trend is expected to continue with better visualization of valvular structures using TEE. Mitral valve pseudopapillary fibroelastoma, a pedunculated mass on the mitral valve occurring with the typical clinical and echocardiographic presentations of a papillary fibroelastoma but lacking the characJOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 87, NO. 1
teristic histologic features, has not been described previously. Generally, papillary fibroelastomas are asymptomatic and are incidental discoveries either during routine echocardiographic evaluations in living patients or at autopsy. Neurologic and cardiac symptoms, including expressive aphasia, monocular blindness, stroke, transient ischemic attack, angina pectoris, and sudden death have been associated with these tumors.1 Fine and Pai5 have shown postmortem evidence of either obstruction or ball valve occlusion of a coronary 69
CASE REPORT
ostium in patients with angina or sudden death attributed to aortic valve papillary fibroelastomas. In the patient described here, a TEE showed a homogeneous pedunculated tumor attached to the anterior leaflet of the mitral valve via a short stalk and moving freely with the mitral valve (Figure 1). The clinical significance of pseudopapillary fibroelastomas is uncertain. However, there could be substantial potential for embolization leading to cardiovascular accident, angina, or sudden death. The pathophysiologic mechanisms leading to the formation of pseudopapillary fibroelastomas also are unknown. We speculate that several pathologic states, including valvular degeneration, true neoplasms, hamartomas, lipomas, or organizing thrombus (as in our patient), may present as pseudopapillary fibroelastomas. Despite excellent resolution and anatomic and morphologic clarification of fibroelastomas using TEE, a definitive diagnosis can be made only after histological staining to differentiate true papillary fibroelastomas from pseudopapillary fibroelastomas. Transesophageal echocardiography is clearly superior to 2-D echo in evaluating valvular mass lesions in view of its superior resolution and absence of interfering structures. Therefore, it should be a logical next approach in attempting a definition of suspicious valvular lesions seen on 2-D echo. In either case, surgical resection of the tumor with valve repair is the best chance for averting adverse consequences that may be associated with these tumors. Literature Cited 1. de Virgilio C, Dubrow TJ, Robertson J, Siegal S, Ginzton L, Nussmeier M, et al. Detection of multiple cardiac papillary fibroelastomas using TEE. Ann Thorac Surg. 1989;48:1 19-121. 2. Shub C, Tajik AJ, Seward JB, Edwards WD, Pruitt RD, Orszulak TA, et al. Cardiac papillary fibroelastomas. Twodimensional echocardiographic recognition. Mayo Clin Proc. 1981;56:629-633. 3. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelastoma and stroke. Echocardiographic diagnosis and guide to excision. Am J Med. 1986;80:129-132. 4. McAllister HA Jr, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology; 1978:1-141. 5. Fine G, Pai SR. Cardiac papillary fibroelastoma: a source of coronary artery emboli and myocardial infarction. Henry Ford Hosp Med J. 1 984;32:204-208.
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A Page in History... The following comments were readers' reactions to the first issue of the Journal in 1909:
I received the journal a couple of days ago. I am very much pleased with it and extend you and Dr Roman, my heart-felt thanks for the energy you have put into its production. It is a very commendable effort and the Association owes to you a lasting gratitude. It looks as well as most of the medical periodicals, we see hereabout. I feel that with such a magnificent pamphlet for a beginning it is bound to grow into a most useful article to the Negro profession. I feel more than proud to have the satisfaction of being able to live long enough to see the first Negro Medical Journal published by our race anywhere in the world. It reminds me that the Biblical historian has said that "Ethiopia would again stretch forth her hand and become a people." 4000 years antedating Hippocrates the Africans were well grounded in the healing art. So it seems that we are about to come back into our own.-P.A. Johnson, MD, President of the National Association, New York, New York I am in receipt of the Journal of the National Medical Association and am much pleased with it. It reflects credit on those to whom its makeup was intrusted.-J.A. Robinson, MD, Darlington, South Carolina
The Journal is all that could be expected and I don't believe that anyone will find any substantial criticism.-W.G. Alexander, MD, Orange, New Jersey The feeling of our members as a whole, is that the first issue of the National Medical Association Journal is a giant success and with the hearty support it is sure to get, it will be one of the foremost publications of our people anywhere; we feel proud of it.-Eugene I. Wright, DDS, Secretary of the Bay State Medical, Dental and Pharmaceutical Association, Boston, Massachusetts
