P1527 Papillary fibroelastomas: diagnostic challenges and clinical and morphologic features

Abstract

Abstract Introduction Papillary fibroelastomas are rare benign primary cardiac tumors that more frequently involve cardiac valves. They are frequently incidentally discovered by echocardiography but may also cause symptoms. Purpose The aim of this study was to characterize several features of histologically confirmed fibroelastomas. Methods Retrospective analysis of patients with echocardiographic suspicion of fibroelastoma between 2009 and 2019 in a single tertiary center. Echocardiography was compared with histology, and echocardiographic, surgical and pathological information about confirmed fibroelastomas was collected. Results 37 patients (P) (54.1% men) with an echocardiographic suspicion and/or histologically confirmed fibroelastoma were included, with a mean age of 58 +- 3 years (min 22, max 82). Echocardiographic report was analyzed in 34P (91.9%), with 32P (94.1%) reporting a likely fibroelastoma and 2P (5.9%) reporting a non-specified mass. 21P (56.8%) had surgery, with 12P (57.1%) having a surgical suspicion of a fibroelastoma, 2P (9.5%) of a mixoma, 1P (4.8%) of a non-specified mass and 6P (28.6%) with undefined suspicion. Of the 21P who had surgery, 66.7% (14P) had a histologically confirmed fibroelastoma, 1P (4.8%) had a mixoma, and 6P (28.6%) had other diagnoses. From the 14P with histologically confirmed fibroelastoma 64.3% had this suspicion by echocardiography and 35.7% had an echocardiogram reporting a non-specified. There was a global concordance between echocardiography and histology in 52.9%. The mean age of confirmed fibroelastoma P was 54 +-5years, and 50% were men. 7P (50%) were asymptomatic, 2 (14.3%) had a stroke, 2 (14.3%) had syncope, 1 (7.1%) had fatigue, 1 (7.1%) had palpitations and 1P had consciousness alteration. In echocardiography most P (71.4%) had only one mass but 1P had 4 different masses. The tumors had a longer axis between 6 and 25mm, with the majority (57.1%) measuring more than 10mm. 12P (85.7%) had valvular fibroelastomas, 50% of these in the aortic valve (3 in non-coronary cusp, 1 in right coronary cusp and 2 non-specified) and 50% in the mitral valve (all in sub-valvular apparatus, involving anterior leaflet, tendinous chord or papillary muscle). 1P had a left ventricular fibroelastoma (apical) and 1P had four masses in the left atrium. Macroscopically 4 lesions had a gelatinous consistency, 2 of them were membranous, 2 were elastic, 2 were friable, 1 was villainous and in 3 of them consistency was not described. The majority (57%) was white, 14% was translucent and in the rest the color was not specified. There was no described recurrence after surgery and there were no deaths registered. Conclusion In this population there was a reasonable concordance between echocardiography and histology, but in some cases the diagnosis was undefined or wrong. 50% of the patients were asymptomatic and the majority had valvular fibroelastomas, but a few had a different location.