Download Incidental finding of a large pulmonary valve fibroelastoma: A case

Eur J Echocardiography (2006) 7, 253e256
Incidental finding of a large pulmonary valve
fibroelastoma: A case report
Marcos Daccarett a,*, Peter Burke b, Souheil Saba a
a
Division of Cardiology, Providence Hospital and Medical Center, 16001 W. 9 Mile Road,
Southfield, MI 48075, USA
b
Department of Internal Medicine, Providence Hospital and Medical Center,
16001 W. 9 Mile Road, Southfield, MI 48075, USA
KEYWORDS
Large fibroelastoma;
Pulmonary valve;
Transesophageal
echocardiogram;
TEE;
Asymptomatic
incidental
Abstract Papillary fibroelastoma (PFE) is an uncommon primary neoplasm of
cardiac origin. They are solitary neoplasms that historically were an incidental
finding at the time of autopsy. With the advent of two-dimensional echocardiography, symptomatic cases have been reported in current literature, thus causing
a paradigm shift in the management of these tumors. Although the majority of PFE
are benign, because of their potential risk for complication related to embolic and
obstructive phenomena, they are now considered hazardous and require tumor
excision even in asymptomatic patients. We report a case of an asymptomatic
incidental large papillary fibroelastoma within the right ventricular outflow tract.
ª 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights
reserved.
Background
Primary intracardiac tumors are extremely rare
with an overall incidence of !0.3%.1 Papillary
fibroelastomas comprise only 8% of all primary
cardiac tumors, of which the vast majority are
benign.2,3 Our understanding of papillary fibroelas-
* Corresponding author. Tel.: C1 248 544 0515.
E-mail addresses: [email protected] (M. Daccarett),
[email protected] (P. Burke), [email protected] (S. Saba).
tomas (PFE) has changed from an innocuous postmortem finding to a potentially life threatening
pathology. Historically, small right-sided PFEs
discovered on routine echocardiogram in asymptomatic patients were observed clinically.4 Twodimensional echocardiography on symptomatic
patients, however, has demonstrated that these
tumors have a tendency for embolization and
obstruction resulting in cerebral vascular accidents,
pulmonary embolism and myocardial infarction.5
It is now suggested that all PFE, regardless of
size and location, should be resected to avoid
embolic or occlusive-related complications.1,6,7 To
1525-2167/$32 ª 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.euje.2005.04.008
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Received 10 March 2005; accepted 27 April 2005
Available online 31 May 2005
254
our knowledge, only four cases of pulmonary valve
PFE have been reported in previous literature. Our
case demonstrates a large right-sided PFE in an
asymptomatic man that historically could have
been observed without intervention, but due to
its potential for life threatening complications
surgical intervention was carried out with excellent outcome.
M. Daccarett et al.
elastin at the core of each frond, coated with
collagen and lined by flat endocardial cellse
confirming the diagnosis of papillary fibroelastoma
(Fig. 3). The patient had an uncomplicated postoperative course and one and half months follow
up revealed no complications.
Discussion
Case report
Figure 1 Transesophageal echocardiogram short axis view of the base of the heart obtained with multiplane
transducer at 40 demonstrating a 1.4 by 1.5 cm mass on the ventricular surface of the pulmonic valve (arrow).
LA Z left atrium, RVOT Z right ventricular outflow tract, AoV Z aortic valve, PV Z pulmonic valve.
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The patient, an asymptomatic 52-year-old AfricanAmerican male with known hypertension and
dyslipidemia, was found to have a large mass in
the right ventricular outflow tract on routine
echocardiogram. Transesophageal echocardiogram
performed for further evaluation, confirmed a mobile, 1.5 cm by 1.4 cm mass attached to the
ventricular aspect of the pulmonic valve above
the right ventricular outflow tract as seen in long
(Fig. 1) and short axes (Fig. 2) of the valve. A
coronary angiogram demonstrated normal coronary anatomy without significant disease. Surgical
resection was recommended due to the tumor’s
large size and mobility, resulting in high risk for
embolization. A transverse arteriotomy of the
main pulmonary artery was performed revealing
a 1.4 ! 1.0 ! 1.3 cm friable, gelatinous mass fixed
onto the ventricular aspect of the pulmonic valve.
Gross and microscopic histopathology revealed
multiple frond-like structures comprising dense
PFE represent 8% of all primary cardiac tumors.
They are the third most common cardiac primary
tumor after myxomas (30%) and lipomas (10%), but
are the most common valvular tumor as up to 90%
occur on the valvular endocardium.2
PFEs have a predilection for the left side of the
heart. The review of current literature demonstrates that symptomatic PFEs are more likely to
be located on the left side of the heart.1,2,8 Similar
investigations confirm that the most commonly
affected valvular site is the aortic valve, followed
by the mitral and tricuspid valves, respectively.
The pulmonic valve is rarely involved.1,2,8 PFEs are
usually small tumors, and it has been reported by
Sun et al.4 that 99% of all PFEs were 20 mm or less.
The mean age of diagnosis was approximately 60
years, although they have been reported in children and teenagers.9e11
The pathophysiology of these tumors remains
unknown. Several possible theories have been
reported including previous endothelial damage
followed by thrombosis and organization, iatrogenic
Large pulmonary valve fibroelastoma: A case report
255
Figure 2 Transesophageal echocardiogram short axis view of the pulmonic valve obtained with multiplane
transducer at 0 demonstrating a spherical mass attached to the pulmonic valve (arrow). LA Z left atrium,
Ao Z aorta, PV Z pulmonic valve.
the brain, heart and lung resulting in stroke,
myocardial infarction or pulmonary embolus.
Therefore, its location as well as size and mobility
determine potential risks and complications.
Even though the majority of cases are asymptomatic because of their potential for life threatening complications, current literature suggests
that even small PFEs should be excised.1,6,7
Figure 3 Histology. Surgical specimen demonstrating multiple papillary fronds with a central core of dense
fibroelastic tissue (arrow), and loose connective tissue with mucopolysaccharides (hematoxylineeosin staining).
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factors and infectious processes related to
cytomegalovirus.12 Cytomegalovirus has been
recovered from PFEs suggesting a viral induction
of the tumor.
PFEs have a tendency to embolize due to their
soft and friable anatomy, often associated with
adherent thrombus.13,14 The common presenting
symptoms are related to embolic phenomena to
256
Surgery is strongly indicated for patients with
embolic events, occlusive-related symptoms due
to left or right ventricular outflow tract obstruction, and as in our case, those with highly mobile
or large tumors.15e18
The aforementioned case demonstrates a rare
neoplasm with an unusual size and location that
underwent successful resection avoiding potential
catastrophic embolic consequences.
M. Daccarett et al.
8.
9.
10.
11.
References
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14.
15.
16.
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18.
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