Papillary fibroelastoma, unusual cause of stroke in a young man: a case report

Abstract

Background: Papillary fibroelastoma is the third most common primary benign tumor with an incidence of up to 0.33% in autopsy series; it accounts for approximately 75% of all cardiac valvular tumors.

Case presentation: We describe a rare case of a 28-Year-old man that while playing football, had a sudden onset of neurological deficit: aphasia, right hemiparesis and right facial numbness. Transthoracic echocardiography (TTE) showed a 10x10 mm mass attached to the anterior mitral valve leaflet. The patient was treated surgically for the prevention of further embolic complications. Histologic examination of the resected mass revealed a papillary fibroelastoma. It is the third most frequent primary cardiac tumor, after myxoma and fibroma, and the most common primary tumor of heart valves. Despite the benign nature of this tumor, it carries very high risk of embolic complications. The successful complete resection of the papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent.

Conclusions: Differential diagnosis of cardiac masses requires clinical informations, laboratory tests, blood cultures and appropriate use of imaging modalities. Papillary fibroelastoma is a potential cause of embolic stroke in the young. The prompt surgical excision of papillary fibroelastoma is curative and the long-term postoperative prognosis is excellent.

Keywords: Cardiac tumors; Cardioembolic stroke; Case report; Cerebrovascular disease; Papillary fibroelastoma.

Fig. 1

Brain Magnetic Resonance shows areas of recent ischemia in the left cerebral hemisphere with more extensive involvement of the putamen, globus pallidus and temporal and parietal cortical-subcortical posterior lobes

Fig. 2

Epiaortic Magnetic Resonance Angiography shows an occlusion at the origin of the left middle cerebral artery

Fig. 3

Transthoracic echocardiogram. The long axis (panel a) and short axis views (panel b) show the hyperechogenous mass (white arrow) attached to atrial side of A3 scallop of the anterior mitral leaflet

Fig. 4

Transesophageal echocardiogram. The intercommissural view (76°) shows a mobile nodular mass, measuring 1 × 1 cm, on the anterior leaflet of the mitral valve

Fig. 5

The Cardiac MRI shows a round mass adhering to the mitral valve

Fig. 6

In the intraoperative view the lesion appears as a white round mass, without a peduncle. The gross specimen of the mass demonstrated gelatinous appearance and smooth surface, with many white papillary fragments with frond-like projections, typical of papillary fibroelastoma