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CASE REPORT
Year : 2018  |  Volume : 28  |  Issue : 4  |  Page : 236-238

Tricuspid papillary fibroelastoma at multimodal imaging


1 Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy
2 Fondazione Giovanni Paolo Ii, Campobasso, Italy
3 Ospedali Riuniti University Hospital, Foggia, Italy
4 GVM Care and Research, Bari, Italy

Date of Web Publication24-Dec-2018

Correspondence Address:
Proff Natale Daniele Brunetti
Department of Medical and Surgical Sciences, University of Foggia, Viale Pinto 1, Foggia 71122
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcecho.jcecho_48_18

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  Abstract 


Papillary fibroelastoma is a rare benign cardiac tumor, second after cardiac myxoma as more frequent cardiac valvular tumor. The therapy is usually based on surgery according to size, mobility, symptoms, and risks of surgery. We report the case of a 67-year old female with transient ischemic attack. Echocardiography showed the presence of a round mobile formation localized on the atrial side of septal leaflets of tricuspid valve, first identified as an endocarditis vegetation. Cardiac magnetic resonance (cMRI) confirmed the presence of the mass and showed an isointense signal in T1-turbo spin echo sequences, hypointense in cine steady-state free precession and in first-pass sequences, and hyperintense in T2 and phase-sensitive inversion recovery with central hypointense core, with a suspected diagnosis of fibroelastoma. The patient underwent cardiac surgery and histology confirmed the presence of fibroelastic tissue with papillary extroflexions compatible with diagnosis of fibroelastoma. The use of cMRI may be useful in the evaluation of the exact position, dimensions, and nature of cardiac masses and fibroelastomas, diagnostic workup, and preliminary assessment before surgery.

Keywords: Cardiac magnetic resonance imaging, fibroelastoma, valve disease


How to cite this article:
Casavecchia G, Gravina M, Zicchino S, Moranti E, Cuculo A, Macarini L, Di Biase M, Brunetti ND. Tricuspid papillary fibroelastoma at multimodal imaging. J Cardiovasc Echography 2018;28:236-8

How to cite this URL:
Casavecchia G, Gravina M, Zicchino S, Moranti E, Cuculo A, Macarini L, Di Biase M, Brunetti ND. Tricuspid papillary fibroelastoma at multimodal imaging. J Cardiovasc Echography [serial online] 2018 [cited 2019 Sep 15];28:236-8. Available from: http://www.jcecho.org/text.asp?2018/28/4/236/248413




  Introduction Top


Papillary fibroelastoma is a rare benign cardiac tumor (7%–9% of benign primary tumors[1]), second after cardiac myxoma as more frequent cardiac valvular tumor.[2] In 85% of cases, it is a valvular disease affecting aortic valve (35%–63%), mitral valve (9%–35%), tricuspid valve (6%–15%), and pulmonary valve (0.5%–8%)[3] and in a very few cases nonvalvular sites, with left ventricular masses documented on the septum[4],[5] and the outflow tract.[6],[7] Fibroelastomas have also been reported on the right ventricular papillary muscles.[1] The atrial side of the mitral valve is more often affected by the disease;[8] and dimensions may vary from 2 to 70 mm.

Fibroelastomas appear as “sea anemones,” after the multiple frond-like projections, especially in saline solution after surgical resection, they are considered as hamartomas with endothelial origin secondary to turbulent high-stress flow and endothelial dysfunction with proliferation. Iatrogenic cardiac trauma and virus-induced tumor genesis have been also proposed. The majority (75%) of fibroelastomas arise from the valvular endothelium and are left sided.[9] Histology shows endothelium surrounding a layer of mucopolysaccharides and an inner vascular core of connective tissue. The amount of collagen, smooth muscle cells, and elastic fibers is variable within the connective tissue matrix. While fibroelastomas growth is very slow, the risk of superficial thrombosis and embolization is significant.[10],[11] Therefore, even though the majority of cases are asymptomatic, cases of myocardial infarction, stroke, pulmonary embolism, retinal artery embolism, heart failure, and ventricular fibrillation have been also reported. Right-sided fibroelastomas are an important and potentially fatal cause of sudden cardiac death, syncope, chest pain, and dyspnea.[9]

The therapy is usually based on the surgery according to size, mobility, symptoms, and risks of surgery.


  Case Report Top


A 67-year old female came to our attention after a recent transient ischemic attack. Echocardiography showed the presence of a round mobile formation of 12 mm × 9 mm localized on the atrial side of septal leaflets of tricuspid valve [Figure 1]a and [Figure 1]b, first identified as an endocarditis vegetation. Cardiac magnetic resonance (cMRI) confirmed the presence of the mass and showed an isointense signal in T1-turbo spin echo sequences [Figure 1]c, hypointense in cine steady-state free precession and in first-pass sequences [Figure 1]d, and hyperintense in T2 and phase-sensitive inversion recovery with central hypointense core [Figure 1]e and [Figure 1]f, with a suspected diagnosis of fibroelastoma.
Figure 1: (a and b) echocardiography showing a 12 mm × 9 mm formation localized on the atrial side of tricuspid valve septal leaflets. Cardiac magnetic resonance confirmed the presence of the mass and showed an isointense signal in T1-turbo spin echo sequences (c), hypointense in cine steady-state free precession and in first-pass sequences (d), and hyperintense in T2 and phase-sensitive inversion recovery with central hypointense core (e and f), with a suspected diagnosis of fibroelastoma

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The patient underwent cardiac surgery that showed the presence of polypoid cardiac mass (15 mm × 10 mm × 10 mm) with a large base of 10 mm localized on the atrial side of septal leaflets of tricuspid valve [Figure 2]a, [Figure 2]b, [Figure 2]c. Histology confirmed the presence of fibroelastic tissue with papillary extroflexions compatible with diagnosis of fibroelastoma.
Figure 2: (a-c) polypoid cardiac mass (15 mm × 10 mm × 10 mm) with a large base of 10 mm localized on the atrial side of septal leaflets of the tricuspid valve

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  Discussion Top


Papillary fibroelastoma is a rare, more often incidentally found, form of benign cardiac tumor, usually affecting left cardiac valves. Its early diagnosis is paramount to avoid complications such as pulmonary or paradox embolism into systemic circulation. Diagnostic workup to exclude myxomas and valvular endocarditis must be careful. Clinical history, characteristic, size, and position are generally useful for differential diagnosis; echocardiography usually shows a small, mobile, pedunculated, or sessile valvular or endocardial mass, which on many occasions flutters or prolapses into the cardiac chambers during systole or diastole. Echodensity of the tumor's central collagen core strongly supports the diagnosis and allows the distinction from other intracardiac tumors, vegetations, or mural thrombi.[11] Furthermore, cardiac computed tomography (CT) may be useful for the diagnosis of papillary fibroelastomas, with excellent spatial resolution; however, shortcomings as contrast medium and radiation exposure remain.[12] The principal limitation of CT is the lack of soft-tissue contrast resolution. For these reasons, cardiac MRI may presently represent the gold standard for preoperative diagnosis of such cardiac mass. cMRI assessment with gadolinium can be used to distinguish thrombi from tumor after the lack of enhancement.[13] Furthermore, cMRI provides an unrestricted spatial approach, high temporal resolution (30–50 ms), and noninvasive tissue characterization based on multiparametric assessment of the chemical microenvironment, thus allowing noninvasive tissue characterization. cMRI, however, may have limitations with small (<10 mm) and highly mobile masses, as fibroelastomas often are.


  Conclusions Top


The use of cMRI may be useful in the evaluation of exact position, dimensions, and nature of cardiac masses and fibroelastomas, diagnostic workup, and preliminary assessment before the surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R, et al. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-31.  Back to cited text no. 1
    
2.
Steger CM, Hager T, Ruttmann E. Primary cardiac tumours: A single-center 41-year experience. ISRN Cardiol 2012;2012:906109.  Back to cited text no. 2
    
3.
Yandrapalli S, Mehta B, Mondal P, Gupta T, Khattar P, Fallon J, et al. Cardiac papillary fibroelastoma: The need for a timely diagnosis. World J Clin Cases 2017;5:9-13.  Back to cited text no. 3
    
4.
Kamdar F, Win S, Manivel JC, Shumway S, Missov E. A rare nonvalvular left ventricular papillary fibroelastoma: A case report. J Cardiol Cases 2014;9:8-10.  Back to cited text no. 4
    
5.
Saxena P, Shehatha J, Naran A, Rajaratnam S, Newman MA, Konstantinov IE, et al. Papillary fibroelastoma of the interventricular septum: Mimicking a cardiac myxoma. Tex Heart Inst J 2010;37:119-20.  Back to cited text no. 5
    
6.
Efthymiou CA, Moorjani N, Livesey SA. Papillary fibroelastoma in the left ventricular outflow tract. Heart 2007;93:590.  Back to cited text no. 6
    
7.
Niino T, Unosawa S. Papillary fibroelastoma of the right ventricular free wall. Case Rep Surg 2014;2014:654641.  Back to cited text no. 7
    
8.
Neerukonda SK, Jantz RD, Vijay NK, Narrod JA, Schoonmaker FW. Pulmonary embolization of papillary fibroelastoma. Arising from the tricuspid valve. Tex Heart Inst J 1991;18:132-5.  Back to cited text no. 8
    
9.
Mastroroberto P, Olivito S, Onorati F, di Virgilio A, Merola S, Renzulli A, et al. Papillary fibroblastoma of tricuspid valve with pulmonary embolization. Asian Cardiovasc Thorac Ann 2006;14:e53-4.  Back to cited text no. 9
    
10.
Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001;103:2687-93.  Back to cited text no. 10
    
11.
Srivatsa SV, Adhikari P, Chaudhry P, Srivatsa SS. Multimodality imaging of right-sided (tricuspid valve) papillary fibroelastoma: Recognition of a surgically remediable disease. Case Rep Oncol 2013;6:485-9.  Back to cited text no. 11
    
12.
Sharma R, Golian M, Shah P, Jassal DS, Shaikh N. Multimodality cardiac imaging of a left ventricular papillary fibroelastoma: A case report. BMC Res Notes 2017;10:25.  Back to cited text no. 12
    
13.
Hoey ET, Shahid M, Ganeshan A, Baijal S, Simpson H, Watkin RW, et al. MRI assessment of cardiac tumours: Part 1, multiparametric imaging protocols and spectrum of appearances of histologically benign lesions. Quant Imaging Med Surg 2014;4:478-88.  Back to cited text no. 13
    


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