Papillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. signal on T2 weighted imaging and signal hyperintensity after administration of gadolinium contrast confirming the fibroelastic nature of the mass. Surgical excision was performed via ventriculotomy and histopathologic examination was pathognomonic of a papillary fibroelastoma. We conclude that transesophageal echocardiography provides high diagnostic certainty in patients with cardiac papillary fibroelastomas and can reliably identify atypical locations of these tumors on nonvalvular surfaces. A multimodality imaging approach is not necessarily indicated in all patients with this condition. Learning objective Papillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. Transesophageal echocardiography readily identifies nonvalvular papillary fibroelastomas based on echogenicity pedunculated nature and characteristic motion and reliably differentiates them from other cardiac masses. A multimodality imaging approach is not indicated in all patients with this condition. Keywords: Papillary fibroelastoma Nonvalvular Transesophageal echocardiography Cardiac magnetic resonance imaging Introduction Primary cardiac tumors are rare with a lifetime incidence of 0.0017-0.02% [1]. Papillary fibroelastomas are the third most common cardiac tumors after myxomas and lipomas and account for less than 10% of all primary cardiac tumors. The valvular endocardium is the preferential attachment site 80% of the time; nonvalvular manifestations remain exceptionally rare [2 3 Whether a multimodality imaging approach offers significant added value compared with echocardiography alone in the diagnostic evaluation of cardiac papillary fibroelastomas is controversial. We illustrate this point with the case of a rare nonvalvular left ventricular papillary fibroelastoma. Case report A 66-year-old Caucasian male with acute onset of memory difficulties was found to have Rabbit Polyclonal to OR10G6. large bilateral basal ganglia infarctions without evidence of cerebrovascular or carotid artery disease on magnetic resonance angiography. A screening transthoracic echocardiogram was unremarkable. However trans-esophageal echocardiography (TEE) demonstrated a highly mobile 0.9 cm × 0.9 cm spherical A 922500 homogenous echo-dense mass adherent to the left ventricular septal wall. Based on its characteristic appearance and echogenicity pedunculated nature and mobility the mass was identified as a papillary fibroelastoma (Fig. 1A and B; Supplementary data: Video clips 1 and 2). Cardiac magnetic resonance (CMR) imaging was performed for further tissue characterization. The mass appeared homogeneously hypo-intense on T2 weighted imaging. Delayed enhancement images after administration of gadolinium shown signal hyperintensity caused by the fibroelastic nature of the mass (Fig. 1C and D). Fig. 1 Transesophageal echocardiography and cardiac magnetic resonance imaging: (A) transesophageal echocardiogram in the remaining ventricular outflow tract view showing a spherical homogenous echo-dense mass measuring approximately 0.9 cm × 0.9 cm arising … Supplementary data associated with this article can be found in the online version at http://dx.doi.org/10.1016/j.jccase.2013.08.012. The A 922500 patient underwent excision of the mass via ventriculotomy. The medical specimen was a friable gelatinous mass that was loosely attached to the remaining ventricular septal wall (Fig. 2A). Cardiac papillary fibroelastoma was confirmed by histopathology. Low magnification hematoxylin and eosin stain shown branching papillae consisting of hypocellular collagenous cores lined by a coating of endocardial cells and loose myxoid matrix (Fig. A 922500 2B). Immunoperoxidase stain for CD34 highlighted the endocardial lining (Fig. 2C); trichrome stain shown the collagenous nature of the cores (Fig. 2D). Fig. 2 Medical pathology and histopathology: (A) medical specimen of gelatinous friable papillary mass resected from your remaining ventricular septum via ventriculotomy A 922500 (B) low power hematoxylin and eosin.