We describe the case of a 50-year-old woman who was admitted to a pheriferal department for heart failure. The echocardiography revealed a small mass measuring about 1.3 × 1.0 cm adhering to the non-coronary cusp of the aortic valve, mild dilated cardiomiopathy and severe biventricular dysfunction. This mass had erroneously been considered a thrombotic lesion, so the patient was treated with thrombolysis and heparin e.v. Only after a transoesophageal echocardiography a tumour cardiac mass was suspected. The diagnosis of fibroelastoma was confirmed by MRI and then from the anatomic and histoligical definition after surgery

A Burke

A Mügge

D Pacini

DG Di Mattia

DM Shaian

Elena Antonioli

Enrico Vizzardi

Ermanna Chiari

FH Edwards

Gregoriana Zanini

H Tanaka

HA Mc Allister Jr

JP Sun

KW Klarich

Livio Dei Cas

M Winkler

PA Araoz

Pompilio Faggiano

RL Zamora

RM Gowda

Savina Nodari

English

PubMed

Springer - Publisher Connector

BioMed CentralCases Journal
ssOpen AcceCase Report
Thrombus or tumor? a case of fibroelastoma  as indicated during 
the submission process
Enrico Vizzardi*, Pompilio Faggiano, Elena Antonioli, Gregoriana Zanini, 
Ermanna Chiari, Savina Nodari and Livio Dei Cas
Address: Section of Cardiovascular Disease, Department of Applied Experimental Medicine, Brescia University, Brescia, Italy
Email: Enrico Vizzardi* - enrico.vizzardi@tin.it; Pompilio Faggiano - enrico.vizzardi@libero.it; Elena Antonioli - el_79@tiscali.it; 
Gregoriana Zanini - gregoriana77@libero.it; Ermanna Chiari - ermanna.chiari@libero.it.it; Savina Nodari - enrico.vizzardi@libero.it; 
Livio Dei Cas - enrico.vizzardi@libero.it
* Corresponding author    
Abstract
We describe the case of a 50-year-old woman who was admitted to a pheriferal department for
heart failure. The echocardiography revealed a small mass measuring about 1.3 × 1.0 cm adhering
to the non-coronary cusp of the aortic valve, mild dilated cardiomiopathy and severe biventricular
dysfunction. This mass had erroneously been considered a thrombotic lesion, so the patient was
treated with thrombolysis and heparin e.v. Only after a transoesophageal echocardiography a
tumour cardiac mass was suspected. The diagnosis of fibroelastoma was confirmed by MRI and then
from the anatomic and histoligical definition after surgery.
Introduction
Cardiac papillary fibroelastoma (PFE) is a rare primary
cardiac neoplasm of unknown prevalence [1]. Since the
introduction of echocardiography, the diagnosis of these
tumours in living patients has been reported sporadically.
The largest report of pathologically confirmed PFE
includes only 17 patients [2]. Echocardiographically, this
entity has been described as a small, well-delineated,
pedunculated mass with a predilection for valvular endo-
cardium [1-3]. Case reports have associated PFE with cor-
onary, cerebral, pulmonary and retinal artery emboli,
although the frequency with which this occurs is not well
established [4,5].
Case presentation
We describe the case of a 50-year-old woman who was
admitted to a pheriferal department for heart failure
(NHYA class III) in mild idiopathic dilated cardiomyopa-
thy. She had chronic atrial fibrillation and hypertension
in anamnesis. On admission to the hospital, the patient's
blood pressure and heart rate were normal. Her oral tem-
perature was 36.5°C. Examination of the head and neck
revealed no abnormalities; the external jugular vein was
distended; no adenopathy was present. Abdominal exam-
ination revealed mild hepatomegaly. Cardiac examina-
tion revealed a systolic murmur at the fourth intercostal
space on the marginal sternal line. The laboratory results
were normal; no increase in inflammatory index. Chest
radiograph showed an enlarged cardiac silhouette and the
ECG showed atrial fibrillation. The patient also under-
went echocardiography, which revealed a small mass
measuring about 1.3 × 1.0 cm adhering to the non-coro-
nary cusp of the aortic valve, mild dilated cardiomiopathy
and severe biventricular dysfunction. In order to the pres-
Published: 8 January 2009
Cases Journal 2009, 2:31 doi:10.1186/1757-1626-2-31
Received: 17 November 2008
Accepted: 8 January 2009
This article is available from: http://www.casesjournal.com/content/2/1/31
© 2009 Vizzardi et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 4
(page number not for citation purposes)
Cases Journal 2009, 2:31 http://www.casesjournal.com/content/2/1/31ence of AF, this mass had erroneously been considered a
thrombotic lesion, so the patient was treated with throm-
bolysis and heparin e.v.
After fibrinolysis, the dimension of this lesion did not
change, so the patient was transferred to our department.
Transthoracic and transoesophageal echocardiography
was performed to define the mass. It showed a highly
mobile, homogeneous echodense mass with a smooth
surface from a pedicle to the closing margins of the non-
coronary aortic cusp. All three aortic cusps had no signs of
thickening and appeared normal as judged by echocardi-
ography. Colour Doppler revealed no valvular incompe-
tence. From echocardiographic findings, a tumour of the
aortic valve rather than a thrombus or a vegetation was
suspected (Figs. 1 and 2). Magnetic resonance imaging
(MRI) was then performed to determine the character of
the tumour tissue. It revealed a mobile nodular mass
which was characterized by a slightly lower signal inten-
sity than the myocardium at MRI with a single-slice
breath-hold segmented turbo fast low-angle shot tech-
nique.
To prevent systemic embolization, the patient underwent
excision of the tumor. The echocardiographic and MRI
findings were confirmed during surgery. A 1-cm papillary
mass was found attached to the noncoronary cusp of the
aortic valve and was treated by simple excision of the
tumour without aortic valve replacement. Histological
examination revealed several fragments covered by flat-
tened cells. The core was composed of dense fibrous tissue
with hyalinization, consistent with a papillary fibroelas-
toma of the endocardium.
In the following days, the patient no longer showed signs
of dyspnoea. An echocardiogram was performed at dis-
charge without evidence of a mass attached to the noncor-
onary aortic cusp.
Discussion
Papillary fibroelastoma (PFE) is a benign, rare, gelatinous
tumour derived from the endocardium; concerning fre-
quency, it corresponds to the third most common primary
intracardiac tumour, preceded by myxomas and lipomas
[6]. Approximately 90% of PFE affects cardiac valves, usu-
ally as a single lesion, on the atrial face of atrioventricular
valves or on any of the sides of semilunar valves. They
rarely occur as single lesions.
Approximately 44% of PFE are found in aortic valves, fol-
lowed by the undertaking of mitral valve in 35% of cases,
tricuspid valves in 15%, and pulmonary valves in 8% [7].
Reports of cases of those tumors have demonstrated
undertaking of all endocardial surfaces, including papil-
lary muscles, tendinous chords, the septum or free walls
of any of the cardiac chambers [8].
The size of described PFE varies from 0.1 to 4 cm, and
most of them are smaller than 1 cm in diameter [9]. The
age of the patients is variable. Most cases are described in
adults over the age of 50, and there is no difference
between the sexes [7,9,10].
PFE is an incidental finding in most cases, although
among symptomatic patients the clinical presentation is
variable and dependent on location, motility and size of
tumour [7-9]. As most of it originates from left chambers,
the most feared complication is systemic embolization,
especially in cerebral or coronary circulation [7-9]. The
most common clinical presentation described is stroke or
transitory ischemic attack. Other described manifestations
Evidence of a mass on the noncoronary aortic cuspFigure 1
Evidence of a mass on the noncoronary aortic cusp.
Evidence of a mass on the noncoronary aortic cuspFigure 2
Evidence of a mass on the noncoronary aortic cusp.Page 2 of 4
(page number not for citation purposes)
Cases Journal 2009, 2:31 http://www.casesjournal.com/content/2/1/31are: angina, myocardial infarction, sudden death, heart
failure, syncope, pulmonary embolism, blindness,
peripheral embolism with renal infarction [7]. In aortic
valve tumour patients, sudden death and myocardial inf-
arction are the most common manifestations.
Electrocardiographic findings are non-specific. Thoracic
radiography may demonstrate signs of increase of cardiac
chambers, pulmonary hypertension or congestion, espe-
cially if the tumour is occluding the mitral valve. Tran-
sthoracic, particularly transoesophageal, echocardiograms
are the ideal method for tumour diagnosis and character-
ization, as they usually show the mass with its varied pro-
portions, motile or not, well-delimited, pedunculated or
sessile, of round, oval or irregular shape. They are mostly
small (99% less than than 2.0 cm). Magnetic resonance
imaging reveals the mass in valve leaflet or cardiac cham-
ber, and the presence of enhancement with gadolinium in
the tumoral mass increases the suspicion level [7]. Cardiac
catheterization does not contribute to the diagnosis.
PFE has a characteristic appearance, resembling a sea
anemone, with multiple ramifications held by a pedicle to
the endocardium. At histological examination, it consists
of an endothelium coat, which covers a connective tissue
matrix with variable amounts of collagen, smooth muscle
cells and elastic fibres [9].
For symptomatic patients, surgical exeresis is the choce of
treatment, in an attempt to preserve valve tissue and func-
tion [11]. Among asymptomatic individuals, surgical pro-
cedure is controversial, as tumoral motility is the
determining factor for surgical indication, for being ? an
independent embolization and death predictor. Surgery is
curative and there is no report of recurrence [7-9]. Most
investigators recommend an aggressive approach to PFE,
especially for lesions of the left side of the heart. Surgical
excision of the PFE is considered to be curative and, in
most cases, the tumour can easily be removed because of
its pedunculation. In the series of Gowda et al. [7], a valve-
sparing procedure was performed in 90% of patients
referred for surgical excision of the cardiac PFE.
Management before a left side isolated lesion includes
surgical removal, when the mass is big and/or movable or
in the precence of patent oval foramen due to the possibil-
ity of paradoxical embolism [12]. When the patient is not
considered for surgical removal of the PFE, long-term oral
anti-coagulation is recommended, although no rand-
omized controlled data are available.
Conclusion
Transoesophageal echocardiography is proving to be
superior to transthoracic echocardiography, not only in
confirming the precence of a cardiac mass but also in
defining its attachment to the underlying tissue, its exact
location and its relation with surrounding structures
[13].
In our case transthoracic echocardiography wasn't be able
to correctly diagnosed fibroelastoma and so it managed to
a wrong diagnosis and so to a wrong therapy. So there is
the extreme necessity to perform always a transoesopha-
geal echocardiography and particularly when a scarce
acoustic window is present at the transthoracic one in
order to give the correct diagnosis and the correct thera-
phy. In our case the probability that the mass could be a
thrombus was very low. There are no definitive features on
TTE that distinguish papillary fibroelastoma from other
intracardiac masses. The sensitivity and specificity of TTE
are in the order of 88.9% and 87.8%, respectively, with an
overall accuracy of 88.4%, when the tumour is larger than
0.2 cm. When the tumour is less than 0.2 cm, the overall
sensitivity of TTE is 61.9%, compared with 76.6% for TEE
[12]. Single papillary fibroelastomas are detected by TTE
in 91.4% of cases, whereas multiple fibroelastomas are
only detected in 8.6%.
Echocardiographic diagnosis is not easy, particularly
when PFE is small in size, when an associated lesion is
masking the tumour, when there is a lack of significant
characteristics to differentiate PFE from degenerative valve
disease, and when the index of suspicion during the exam-
ination is insufficient [12]. Differential diagnoses include
thrombi, infective or noninfective vegetations, degenera-
tive valve tissue and other cardiac tumours: clinical data,
blood cultures and laboratory tests, together with echocar-
diography [12].
However, it is almost impossible to define tissue character
using this tecnique alone, as in our case. Magnetic reso-
nance imaging excels at making a differential diagnosis of
various soft tissues and organs because of its high resolv-
ing power [14]. Cardiac MRI gives superior visualization
of cardiac tumours, and of metastasis if present [15].
We have presented a case of fibroelastoma that was ini-
tially incorrectly diagnosed by transthoracic echocardiog-
raphy; it was detected and diagnosed by TTE
echocardiography and magnetic resonance imaging. In
cases of cardiac and valvular tumour, transoesophageal
echocardiography and magnetic resonance imaging play
important roles in making a diagnosis and selecting the
optimal surgical treatment.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.Page 3 of 4
(page number not for citation purposes)
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Competing interests
The authors declare that they have no competing interests.
Authors' contributions
EC and PF performed transthoracic and thransesophageal
echocardiography. EV, GZ and EA treated and follow-up
the patient during the admission and the stay in the hos-
pital. SN and LDC gave us the supervision.
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Thrombus or tumor? a case of fibroelastoma as indicated during the submission process

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Livio Cas

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Tumors of the heart and great vessels.

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Thrombus or tumor? a case of fibroelastoma  as indicated during the submission process

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