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Case Reports
. 2011 Mar;4(3):307-11.
Epub 2011 Mar 22.

Left atrial myxoma with papillary fibroelastoma-like features

Abbas Agaimy  1 , Thomas Strecker
Affiliations
Case Reports

Left atrial myxoma with papillary fibroelastoma-like features

Abbas Agaimy et al. Int J Clin Exp Pathol. 2011 Mar.

Abstract

Although rare, papillary fibroelastoma (PFE) of the heart valves and atrial myxoma represent the two most common cardiac tumors. Coexistence of these two lesions has been documented in rare case reports. We describe the case of a 77-year-old man who presented with slightly progressive chest pain associated with dyspnea, fatigue and edema of the lungs. Transthoracic echocardiography detected a left atrial mass that has been successfully excised. Histopathological examination showed a neoplasm combining features of both atrial myxoma and PFE. However, close evaluation of the latter showed microscopic foci of myxomatous tissue within papillary cores, indicating that the PFE-like component has developed around preexisting myxomatous tissue that served as a nidus for papillary fronds, probably by a process of fibrinous microthrombosis, organization and endothelialisation. This unusual case may shed light on the pathogenesis of the PFE pattern.

Keywords: Papillary fibroelastoma; atrium; calretinin; myxoma.

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Figures

Figure 1
Figure 1
Transesophageal echocardiography revealed large heterogeneous, lobulated echogenic mass attached to the interatrial septum (LA, left atrium).
Figure 2
Figure 2
Intraoperative gross photograph showed a gelatinous tumor mass (maximal diameter of 24 × 21 mm).
Figure 3
Figure 3
(A) Histological examination showed typical myxomatous tissue (lower left) adjacent to papillary frond-like structures very reminiscent of papillary fibroelastoma (upper right, note intervening thrombotic material). (B) Papillary structures alternating with hyperchromatic myxoma cells, note myxomatous cores within some papillae. (C) Elastica van Giesson stain in this area of the tumor was indistinguishable from PFE with some darkly staining granular elas-totic material. (D) Higher magnification of the papillae showed minute myxomatous foci (note residual bleeding and fibrin in some papillae). (E) The tumor in other areas displayed classical myxomatous appearance and expressed strongly calretinin (subimage). (F) Calretinin immunostaining highlighted myxoma cells covering the papillae and within papillary cores (note non-staining flat endothelial cells covering the papilla on the left).
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References

    1. Burke AP, Virmani R. Atlas of Tumor Pathology, third series, fascicle 16. Washington, DC: Armed Forces Institute of Pathology; 1996. Tumors of the heart and great vessels.
    1. Blondeau P. Primary cardiac tumors. French studies of 533 cases. Thorac cardiovasc Surg. 1990;38:192–195. - PubMed
    1. Raeburn C. Papillary fibro-elastic hamartomas of the heart valves. J Pathol Bact. 1953;65:371–373. - PubMed
    1. Strecker T, Agaimy A, Marwan M, Zielezinski T. Papillary fibroelastoma of the aortic valve: appearance in echocardiography, computed tomography, and histopathology. J Heart Valve Dis. 2010;19:812. - PubMed
    1. Prifti E, Bonacchi M, Salica A. Mitral valve myxoma concomitant with papillary fibroelastoma. Ann Thorac Surg. 2000;70:335–6. - PubMed

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