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Recurrent Cardiac Fibroelastoma. Is It Really a Benign Tumor?

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Papillary fibroelastomas are the second most common cardiac tumor. These tumors are benign and typically occur in the valvular endocardium, and most present as solitary masses. Only a small percentage… Click to show full abstract

Papillary fibroelastomas are the second most common cardiac tumor. These tumors are benign and typically occur in the valvular endocardium, and most present as solitary masses. Only a small percentage cause symptoms. Recurrence of these tumors is exceptional–until now it has not been described in the literature–and the treatment for such cases is unclear. We present the case of a 32-year-old man who presented with a stroke. Transthoracic echocardiography showed a mobile mass attached to the mitral valve causing mild regurgitation, with no other abnormalities. Three-dimensional transesophageal echocardiography (3D-TEE) confirmed the presence of a 6 5 mm round tumor, with a friable appearance, attached to the atrial aspect of the mitral valve, at the free edge of the posterior leaflet (P1), suspicious of papillary fibroelastoma (Figure 1). In addition, electrocardiography showed negative T-waves in the inferior leads, and consequently magnetic resonance was performed, which showed a small midsegment-basal subendocardial infarct of the posterior septum and inferior wall (Figure 1). With this suspected emboligenic papillary fibroelastoma, the patient underwent cardiac surgery, in which the mass was resected, with valvuloplasty using a pericardial patch. The surgical result was good, with no residual regurgitation. Pathology showed papillary fronds of connective tissue with few cells, covered with a single layer of endothelial cells, findings characteristic of a papillary fibroelastoma (Figure 1). At follow-up, the patient remained asymptomatic, and transthoracic echocardiograms performed every 6 months showed no abnormalities. At 2 years postintervention, the patient had sudden-onset low back pain, and renal embolism was diagnosed. Repeat 3D-ETT was performed, which showed a new 4 4 mm tumor, with similar characteristics, this time located on the atrial aspect of the anterior leaflet of the mitral valve (A1), just in front of the location of the previous mass (Figure 2). Given this rapid tumor recurrence and the aggressive presentation on both occasions (stroke, myocardial infarction, and renal embolism), it was decided to perform mechanical mitral valve replacement (Figure 2). Pathology again confirmed that this was a fibroelastoma. After myxoma, papillary fibroelastoma is the second most common benign cardiac tumor. These tumors originate in the valvular endocardium, mainly in the aortic and mitral valves, although cases have also been described of attachment to the ventricular walls. They usually present as solitary masses or, rarely,

Keywords: fibroelastoma; figure; pathology; mitral valve; papillary fibroelastoma; tumor

Journal Title: Revista espanola de cardiologia
Year Published: 2018

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