A giant benign epicardial lipomatosis of the left ventricle | |
DOI: 10.5606/e-cvsi.2016.587 | |
Trushar Gajjar, Nageswar Rao, Neelam Desai | |
Sri Sathya Sai Institute of Higher Medical Sciences - Prasanthigram, CTVS Department, Prasanthigram, Andhra Pradesh, India | |
Keywords: Benign cardiac tumor; cardiac malignancy; epicardial lipomatosis; left ventricle | |
Fatty masses of the heart are relatively uncommon. Herein, we report an extremely rare case of a giant benign epicardial lipomatosis arising
from the left ventricle. A 26-year-old female presented with atypical chest pain and easy fatigability for the past six months. Chest X-ray
showed an increased cardiothoracic ratio. Echocardiography revealed a large homogenous mass in the pericardial space near the lateral
surface of the left ventricle. Computed tomography and magnetic resonance imaging of the chest showed a large fat-density mass with
well-defined margins, arising from the left ventricle. The patient underwent surgical removal of the mass without cardiopulmonary bypass.
She recovered well and discharged in the seventh postoperative day. |
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Primary cardiac tumors account for 5 to 10% of all
neoplasms of the heart and pericardium with an
incidence ranging from 0.0001 to 0.05% in autopsies.[1]
Lipomas are the second most common benign cardiac
tumors.[2] These tumors are often asymptomatic in
nature and usually detected incidentally, mostly during
autopsies.[1-3] Although a lipoma is a benign tumor, it
can lead to compression of cardiac chambers and cause
life-threatening complications, when the tumor size
increases.[1-6] Herein, we report a rare case of a giant
benign epicardial lipomatosis arising from the left
ventricle. |
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CASE PRESANTATION
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A 26-year-old female had atypical chest pain and
easy fatigability for the past six months. Chest
X-ray showed a cardiothoracic ratio of 70%.
Echocardiography (ECHO) showed a large
homogenous, echogenic mass in the pericardial
space located along the lateral surface of the
heart, arising from the epicardium and extending
posterior, anterolateral surfaces of the left ventricle
(LV) including the apex with minimal pericardial
effusion (Figure 1a). The biventricular function was
good with no regional wall motion abnormalities.
Computed tomography (CT) of the chest showed
a large fat-density mass, extending from the
arch of the aorta superiorly to the dome of the
diaphragm inferiorly in the left hemithorax. The mass had a well-defined lateral margin limited by
the pericardium peripherally and its medial border
was encasing the left atrium, LV and a part of right
ventricle (RV). The mass also encased the left
anterior descending artery and derived its blood
supply from the branches of the left coronary artery
(Figure 1b). The tumor was found to be causing a
mass effect on the left lower lobe of the lung and its
vasculature. Cardiac magnetic resonance imaging
(MRI) showed an abnormal soft tissue enhancement
in the anterolateral surface of the heart occupying
the anterolateral, posterior and inferior surfaces
of the LV and anteriorly extending over the RV
(Figure 1c). The mass was well-defined and had
the same signal intensity as subcutaneous fat, in
all the images and occupying almost the whole of
the left hemithorax (Figure 1d). The mass was in
close contact with the cardiac chambers, but not
compressing any chambers or great vessels.
Surgical operation |
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Benign tumors of the heart were rare. Lipomas
are the second most common benign cardiac
tumors.[2] Cardiac lipomas represent about 10% of
all cardiac tumors and are thought to originate either
from the subendocardium, subpericardium or the
myocardium.[3] The majority of the cardiac lipomas
are subendocardial or epicardial, and only 25% are
found in the myocardium.[1-4] Clinical presentation
usually depends on the size and location of the
tumor. They usually originate from the epicardial
fat tissue and grow slowly into the pericardial
sac; in case of asymptomatic cases, they often
achieve enormous dimensions before producing any
symptoms.[1-4] Many tumors are clinically silent,
being found only during necropsy or on routine
chest X-rays.[1-4] These tumors cause symptoms
through compression of the heart. Anginal pain due
to coronary arterial compression, atrial arrhythmias, and effort intolerance has been also reported.[4]
The subendocardial tumors with an intracavitary
extension may cause symptoms characteristic of
their location; they usually produce early symptoms,
such as congestive heart failure, supraventricular or
ventricular arrhythmias, syncope, or sudden cardiac
death.[5] Chest X-ray usually does not differentiate
between pericardial effusion and a subepicardial
lipoma. In addition, ECHO accurately defines the
location and extent of the mass.[1-4] However, the
acoustic appearance of an intra-pericardial lipoma is
not diagnostic. Subepicardial lipomas usually appear
relatively ECHO-lucent and may be erroneously
interpreted as the pericardial fluid.[4] Also, CT
and MRI can demonstrate lipomatous tumors.[1-4]
The latter can distinguish between lipomas and
other tumors, such as liposarcomas. It also provides
a clear definition of blood vessels to precisely
detect the location, size, and extension of the
tumor.[1-4] It also demonstrates the relationship of
the tumor to the coronary arteries[1] and estimates
the intramyocardial extension. These are important factors in determining tumor resectability and help
planning the management approach.[4] Although
some epicardial tumors may be removed without
CPB, most intramural and intracavitary tumors
should be excised with the use of CPB, as cardiac
manipulation may release part of the tumor, causing
an embolism. Following the first successful removal
of an intrapericardial lipoma by Maurer in 1952,
several sporadic cases have been reported.[1-6] Benign
lipomas can be mostly excised completely with low
morbidity and mortality and with excellent long-term
results.[1] In the majority of cases, intrapericardial
lipomas may be removed without CPB. However,
CPB may be required in cases of recurrence and
when there is an intracardiac extension. The decision
to resect such epicardial lipomatosis is going to be
debulking, as it is densely adherent to the heart and
major coronary arteries. Off pump debulking allows
ease of identifying bleeders which is going to be a
much more difficult job on the pump, since the heart
is arrested and bleeders would not be seen clearly.
Intraoperatively, it is important to excise the entire tumor along with the pedicle to prevent recurrences.
The rate of lipoma recurrence after total and subtotal
resection is very low.[6] Our case seems to be
exceptional, as this was a giant benign lipomatous
tumor without any pedicle directly arising from the
LV surface. To the best of our knowledge, this is the
first case reported in the literature. In conclusion, most of the patients with intrapericardial lipomas are asymptomatic for a long time due to the slow growth of the tumor. Imaging modalities such as computed tomography and magnetic resonance imaging are the main diagnostic tools. Surgical resection is necessary to prevent tumor compression syndromes of the heart. Finally, the status of malignancy needs to ruled out and confirmed by histological examination.
Declaration of conflicting interests
Funding |
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1) Miralles A, Bracamonte L, Soncul H, Diaz del Castillo R, Akhtar
R, Bors V, Pavie A, et al. Cardiac tumors: clinical experience and
surgical results in 74 patients. Ann Thorac Surg 1991;52:886-95.
2) Burke A, Virmani R. Tumors of the heart and great vessels.
In: Atlas of tumor pathology. Washington: Armed Forces
Institute of Pathology; 1996.
3) Hananouchi GI, Goff WB 2nd. Cardiac lipoma: six-year
follow-up with MRI characteristics, and a review of the
literature. Magn Reson Imaging 1990;8:825-8.
4) Doshi S, Halim M, Singh H, Patel R. Massive intrapericardial
lipoma, a rare cause of breathlessness. Investigations and
management. Int J Cardiol 1998;66:211-5.
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