A rare nonvalvular left ventricular papillary fibroelastoma: A case report

Abstract

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. We report the case of a 66-year-old Caucasian male with acute bilateral basal ganglia infarctions found to have a mass adherent to the left ventricular septum by transesophageal echocardiography. The mass was identified as a rare nonvalvular cardiac papillary fibroelastoma based on echogenicity, pedunculated nature, and typical motion. Tissue characterization by cardiac magnetic resonance imaging demonstrated homogeneously hypo-intense 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: Cardiac magnetic resonance imaging; Nonvalvular; Papillary fibroelastoma; Transesophageal echocardiography.

Fig. 1

Transesophageal echocardiography and cardiac magnetic resonance imaging: (A) transesophageal echocardiogram in the left ventricular outflow tract view showing a spherical, homogenous, echo-dense mass measuring approximately 0.9 cm × 0.9 cm arising from the left ventricular septum (arrow), (B) magnified view of the left ventricular septal mass, (C) cardiac magnetic resonance delayed enhancement images in the long axis and (D) short axis showing signal hyperintensity of left ventricular septal mass after gadolinium administration (arrow).

Fig. 2

Surgical pathology and histopathology: (A) surgical specimen of gelatinous, friable, papillary mass resected from the left ventricular septum via ventriculotomy, (B) low power hematoxylin and eosin photomicrograph demonstrating branching papillae consisting of hypocellular collagenous cores lined by a layer of endocardial cells and loose myxoid matrix, (C) immunoperoxidase stain for CD34 highlights the endocardial lining and (D) trichrome stain highlights the collagenous nature of the cores.