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Ann Thorac Surg 2000;70:335-336
© 2000 The Society of Thoracic Surgeons
a Istituto di Chirurgia del Cuore e dei Grossi Vasi, Università degli Studi di Roma, "La Sapienza", Policlinico Umberto Primo, Viale del Policlinico, 155, 00161, Rome, Italy
e-mail: edvinprifti{at}hotmail.com
To the Editor
We read with interest the article by Howard and coworkers [1]. Papillary fibroelastoma is still extremely uncommon and most frequently arises from valvular endocardium. Almost 97 cases of papillary fibroelastomas have been reported in the literature [1]. Myxoma is the most common cardiac neoplasm of adults occurring mostly in the left atrium, generally in the region of the fossa ovalis. A myxoma arising from the mitral valve (MV) is an exceedingly rare lesion, with only 8 reported cases in the literature [2]. We report here a case of MV myxoma coincident with papillary fibroelastoma.
A 42-year-old woman was admitted for dyspnea, fever, and arthralgias. Physical examination revealed an apical systolic-diastolic rumble. Transthoracic echocardiography revealed a mobile, heterogeneous mass originating from the atrial surface of the anterior MV leaflet. Transesophageal echocardiography confirmed the transthoracic echocardiographic findings and revealed a mobile echogenic density mass located on the ventricular surface of the anterior MV leaflet, at the junction of the chordae tendinae. Both examinations revealed severe MV regurgitation. At operation a 3 x 3.5-cm broad-based fleshy mass was found originating from the atrial surface of the anterior MV leaflet. Another mass of 0.6 x 1 cm, with a smooth, rubbery surface and yellow-white color, was identified on the ventricular surface of the anterior MV leaflet, between the chordae tendinae. The tumoral masses and anterior MV leaflet were excised and the patient underwent MV replacement. The diagnosis of myxoma was confirmed by histology. Special stains (Masson/trichrome for collagen, Van Gieson for elastic fibers, and periodic acid schiff (PAS) staining), and immunohistochemistry were performed. Histologic examination of the tumoral mass found on the ventricular surface of the anterior MV leaflet revealed a rim of elastic fiber and a myxoid matrix with collagen fibers. The outer layer consisted of regular endothelial cells; therefore, we concluded that the mass was a papillary fibroelastoma.
The first case of MV myxoma was reported in 1979 [3], coinciding with the first reported case of papillary fibroelastoma founded incidentally during an operation for ventricular septal defect repair [4]. Transesophageal echocardiography is a valuable technique in the preoperative diagnosis of cardiac tumors, offering significantly greater anatomic detail regarding size and site of attachment of the masses. This information is critical to the surgeon in planning the operative procedure as well as postoperative assessment.
Mitral valve myxomas present in much the same way as left atrial myxomas. Progressive or acute MV dysfunction may be the presenting feature. As with myxomas of the left atrium, MV myxomas carry the tendency for embolization with transient ischemic attack and stroke. In 7 of the 8 previously reported cases the myxoma originated from the atrial side of the MV leaflet, as it did in our case.
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. Lee and colleagues [5] described an atypical presentation of papillary fibroelastoma mimicking multiple vegetations in suspected subacute bacterial endocarditis. In our case, initially it was hypothesized to be a postrheumatic endocarditis, and the mass on the ventricular side of anterior MV leaflet was thought to be a vegetation. Treatment, as with all myxomas or papillary fibroelastoma, is surgical excision. Smaller lesions, or those arising from a stalk, may be resected without the need for valve replacement. Frequently, as in the case presented, however, the remaining defect in the valve leaflet necessitates valve replacement.
References
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  T. Menon, Y. Watanabe, and D. Andrews Concurrent primary cardiac tumors J. Thorac. Cardiovasc. Surg., July 1, 2007; 134(1): 263 - 264. [Full Text] [PDF]
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