Circulation. 2007;115:e3-e6
doi: 10.1161/CIRCULATIONAHA.106.637132
(Circulation. 2007;115:e3-e6.)
© 2007 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Papillary Fibroelastoma of the Aortic Valve
Appearance in 64-Slice Spiral Computed Tomography, Magnetic Resonance Imaging, and Echocardiography
Alexander Lembcke, MD;
Rudolf Meyer, MD;
Dietmar Kivelitz, MD;
Holger Thiele, MD;
Christian Barho, MD;
Johannes M. Albes, MD;
Holger Hotz, MD
From the Department of Radiology, Charité–Universitätsmedizin Berlin, Berlin, Germany (A.L.); Department of Cardiothoracic and Vascular Surgery, Laboratory of Pathology, Deutsches Herzzentrum Berlin, Berlin, Germany (R.M.); Departments of Radiology (D.K.) and Cardiology (H.T.), Herzzentrum Leipzig, Leipzig, Germany; Kardiologische Praxis Neubritzer Tor, Berlin, Germany (C.B.); Department of Cardiovascular Surgery, Herzzentrum Brandenburg, Bernau, Germany, (J.M.A.); and Cardiocentrum Berlin, Berlin, Germany (H.H.).
Correspondence to Alexander Lembcke, MD, Institut für Radiologie, Charité–Universitätsmedizin Berlin, Campus Charité Mitte, Charitéplatz 1, 10117 Berlin, Germany. E-mail alexander.lembcke{at}gmx.de
A 60-year-old man with an acute stroke of unknown origin was referred to our institution for further diagnostic evaluation. Contrast-enhanced ECG-gated 64-slice spiral computed tomography depicted a well-defined, pedunculated, mobile, spherical lesion (density 69±21 Hounsfield units) attached to the commissure of the left coronary and noncoronary aortic valve leaflet. The lesion showed a relatively homogenous inner structure with a slightly villous outer margin (Figure 1A through 1D). ECG-gated magnetic resonance imaging exhibited a solid lesion with intermediate signal intensity on both T1- and T2-weighted spin-echo sequences (Figure 2A and 2B). Transthoracic and multiplane transesophageal echocardiography also demonstrated a spherical solid structure within the aortic root, confirming the computed tomography and magnetic resonance imaging findings (Figures 3A and 3B and 4A through 4C). Dynamic computed tomography files (using the temporal information of the recorded 4D computed tomography dataset), cine magnetic resonance imaging (using dynamic steady-state free-precession gradient-echo sequences), and color-coded Doppler echocardiography demonstrated a competent aortic valve with a highly mobile, slightly deformable intraluminal mass (Movies I through IV). Surgery was performed and the tumor was completely removed. The gross pathological specimen appeared as a translucent, gelatinous mass (Figure 5A). Histological examination revealed a benign tumor with multiple papillary fronds of different size that consisted of an acellular matrix surrounded by a single layer of endothelial cells, leading to the definite diagnosis of papillary fibroelastoma (Figure 5B and 5C).
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Figure 1. Contrast-enhanced ECG-gated multislice spiral computed tomography. Color-coded, semitransparent, 3-dimensional reconstruction in angulated coronal (A), transverse (B), and sagittal (C) planes and color-coded endoluminal view (D) demonstrating a well-defined, pedunculated, spherical mass (arrows) located in both the left and noncoronary sinus slightly above the aortic valve.
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Figure 2. ECG-gated magnetic resonance imaging. T1-weighted (A) and T2-weighted (B) spin-echo sequences showing a solid lesion (arrows) with intermediate signal intensity.
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Figure 3. Transthoracic echocardiography (A) and multiplane transesophageal echocardiography (B) depicting a spherical echogenic structure (arrows) within the aortic root.
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Figure 4. Submillimeter, multiplanar, reformatted computed tomography image across the aortic root (A) in direct comparison with corresponding images obtained with a steady-state free-precession gradient-echo magnetic resonance imaging sequence (B), and multiplane transesophageal echocardiography (C), all demonstrating a solid, intraluminal mass (arrows) attached to the commissure of the left coronary and noncoronary aortic valve leaflet.
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Figure 5. A, The macroscopic specimen appeared as a translucent, gelatinous mass. B, The histological specimen in the hematoxylin & eosin stain (magnification x10) shows a benign lesion with multiple papillary fronds. C, The papillary fronds consist of 3 layers comprising a collagenous core with low elastin content, an amorphous intermediate layer, and a delicate coat of single-layer endothelium as demonstrated in elastica–van Gieson stain (magnification x50).
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Acknowledgments
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Disclosures
None.
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Footnotes
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The online-only Data Supplement, consisting of movies, is available with this article at http://circ.ahajournals.org/cgi/content/full/115/1/e3/DC1.
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