European Journal of Echocardiography Advance Access published online on May 29, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen164
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
Primary cardiac angiosarcoma evaluated with contrast two-dimensional and real-time three-dimensional echocardiography
Hyun Suk Yang,
Shantanu Sengupta,
Matt M. Umland,
Krishnaswamy Chandrasekaran and
Farouk Mookadam*
Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, AZ 85259, USA
Received 6 February 2008; accepted after revision 12 April 2008.
* Corresponding author. Tel: +1 480 301 6907; fax: +1 480 301 8018. E-mail address: mookadam.farouk{at}mayo.edu
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Abstract
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A 63-year-old lady presented with syncope and atypical chest
pain. Conventional two-dimensional (2D) contrast echocardiogram
showed a highly vascular mass compressing the right atrium without
direct communication. A real-time three-dimensional echocardiogram
(RT3DE) revealed its outer spatial relationship with the surrounding
structures and its inner heterogeneity as well. A chest computed
tomography revealed a solid cardiac mass (6
x 7 cm
2)
arising from the right atrium and multiple pulmonary nodules.
Histopathology of the pericardial fluid confirmed angiosarcoma.
To our knowledge, this is the first report of histology-confirmed
primary cardiac angiosarcoma, which was completely evaluated
by conventional 2D, contrast, and RT3DE.
Keywords: Angiosarcoma; Contrast echocardiography; Three-dimensional echocardiography
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Introduction
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Primary cardiac sarcomas are extremely rare.
1 At the time of
discovery, up to 75% have systemic metastases,
2 and hence a
poor prognosis with a median survival of 3–6 months.
3 Echocardiography is a reliable, non-invasive, and widely available
tool for detecting cardiac tumours. Although a final diagnosis
depends on histopathologic confirmation, a complete echocardiogram
is essential to help define the site, size, and location of
the mass in relation to cardiac anatomical structures and to
assess the presence of pericardial fluid and the haemodynamic
effects thereof. Furthermore, the use of contrast echocardiography
is beneficial in assessing the vascularity of the tumour and
evidence of contiguous invasion. More recently, the use of real-time
three-dimensional echocardiography (RT3DE) can be helpful in
evaluating the volume and spatial relationship with adjacent
structures. This report describes a complete echocardiographic
evaluation of a patient with a rare primary cardiac angiosarcoma
using the conventional 2D, perfusion contrast imaging, and RT3DE.
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Case
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A 63-year-old female presented with a history of syncope and
atypical chest pain. She accumulated 45 pack years of tobacco
with no other cardiovascular risk factors. Cardiac examination
revealed distant heart sounds, an ejection systolic murmur at
the base, and an intermittent biphasic pericardial rub. A notable
abnormality in blood testing was a hypochromic microcytic anaemia.
The chest radiograph (
Figure 1) showed an increased cardiothoracic
ratio and increased bronchovascular markings. Two-dimensional
transthoracic echocardiography was performed using the conventional
2D probe (4V1c) of the Acuson C512 ultrasound system (Siemens
Medical Solutions USA, Inc., Mountain View, CA, USA). It revealed
normal left ventricular size, systolic and diastolic function.
Subcostal imaging revealed a large cardiac mass measuring 8
x 4 cm
2 adjacent to the right atrium (
Figure 2)
with a small loculated pericardial effusion with focal thickening
and oedema adjacent to mass. To assess the vascularity of this
mass, a single bolus of 0.1 ml perflutren lipid microsphere
(Definity
TM, Bristol-Myers Squibb Medical Imaging, North Billerica,
MA, USA) was administered intravenously. Contrast images were
acquired with the ultrasound system set to a lower mechanical
index (MI = 0.3) and a lower decibel power (–16 dB).
Initial bolus passage of the contrast demonstrated a clear demarcation
between the mass and right atrial cavity without a direct communication
between the mass and the right heart chambers (
Figure 3,
see Supplementary Movie I); 10 cardiac cycles later, an enhanced
flow stream appeared within the mass, and revealed this to be
a highly vascular mass, with no evidence for communication with
either the right atrium or ventricle (
Figure 4,
see Supplementary Movie II).
The perfusion contrast intensity of the solid part of the mass
was higher than that of the adjacent myocardial tissue, and
less than the signal from the cystic part of the mass or the
right atrial blood pool which was consistent with a highly vascular
mass, whereas the 10 cycle delayed enhancement showed the vascularity
and blood pool to be independent of the cardiac chambers. These
characteristics would define an angiosarcoma. We gained further
spatial information about this mass via RT3DE, which images
revealed the mass originating from the lateral wall of the right
atrium, extending to the pericardium. The mass compressed the
right atrial cavity without any obstruction of the inferior
vena cava and no communication with the right-sided cardiac
chambers. Real-time three-dimensional echocardiography defines
the mass as having mixed echogenicity, predominantly cystic
with fluid with some hyperechoic densities consistent with a
vascular tumour (
Figure 5,
see Supplementary Movie III)
with a small loculated pericardial effusion. Chest computed
tomography (CT) with contrast revealed a heterogeneous mixed
signal enhancing cardiac mass measuring 6
x 7 cm
2 and abutting the superior vena cava and right atrium with associated
pericardial effusion (
Figure 6). There was no mediastinal
mass; however, there were several ground-glass lesions scattered
bilaterally suggestive of pulmonary metastasis (
Figure 7).
Histopathology of the cell block after biopsy of the pericardial
fluid demonstrated angiosarcoma with positive immunostains for
vimentin, CD31, and CD34. After the CT findings and the histopathologic
conclusions, this was deemed to be a metastatic angiosarcoma
of high grade, and palliative management was recommended.
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Figure 5 Real-time thre-dimensional echocardiogram demonstrates a large mass (arrows) broadly attached to the lateral wall of the right atrium (RA) and the basal lateral wall of the right ventricle, and extends into the pericardium. Heterogeneous inner mass structures are easily visible: the inner multi-lobular tissue density based on the side of pericardium and the surrounding cystic echo-density. The spatial relationship between the large mass and the adjacent structures is well demonstrated. There is no protrusion of the tissue into the RA, and no extension into the inferior vena cava or hepatic vein.
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Figure 6 Chest computed tomography with contrast demonstrates a solid mass (asterisk) arising from the lateral wall of the right atrium, measuring 6 cm transverse, 7 cm anteroposterior, and 9 cm craniocaudad. Most of the mass is located outside of the right atrium (RA), causing slight compression of the RA and right ventricle. This lesion can be an angiosarcoma arising from the heart, a malignant thymoma invading the pericardium, lymphoma, or metastatic tumour. LV, left ventricle; LA, left atrium.
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Discussion
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The frequency of primary tumours has been reported as 0.02 %
in a series of autopsies with the majority being benign.
4 Angiosarcoma
is the most common primary cardiac malignant tumour and is extremely
rare with a postmortem incidence of 0.0001%.
1 It is more common
among males between the third and fifth decade of life.
5–8 In contrast to benign tumours which are usually located in the
left atrium, malignant tumours are found almost exclusively
in the right heart, particularly in the right atrium with a
high proportion of pericardial involvement.
5,9 Primary cardiac
sarcoma seldom causes symptoms until late in its course. Because
of the involvement of the right atrium, primary cardiac angiosarcoma
can often present with chest pain, obstruction to filling of
the right side of the heart, pericardial effusion, dyspnoea,
cardiomegaly, and hepatomegaly.
5–9 These non-specific
symptoms at presentation, coupled with its rarity and rapid
growth, are responsible for the late diagnosis in almost all
reported cases.
Imaging techniques have an important role in establishing the diagnosis. Conventional 2D transthoracic echocardiography has been an important non-invasive tool that helps locate the tumour, define its extent, and may accurately predict tumour type.9 Two-dimensional echocardiographic findings which suggest malignancy include location in the right-sided chambers or intramural lesions and invasion into the vena cava or pulmonary veins. Recurrent pericardial effusion is one of the common clinical features of cardiac angiosarcoma and can result in pericardial constriction.10 Although it is unusual for a benign tumour to be located in right atrial wall and extended to the pericardium, this alone does not predict malignancy. Contrast echocardiographic perfusion imaging is an important supplement to help characterize the vascularity of a cardiac mass: compared with the adjacent myocardium, malignant and vascular tumours hyper-enhance with the use of contrast imaging, whereas stromal tumours and thrombi hypo-enhance.11–13 As the most common primary cardiac malignant tumour, cardiac angiosarcoma is notorious in its gross appearance for being a large bloody mass. This highly vascular mass can now be diagnosed with some confidence using contemporary ultrasound technology combined with contrast perfusion imaging and complemented by RT3DE. There is no published study to our knowledge with a pre-biopsy study using contrast enhancement of a mass with myocardial perfusion suggesting an angiosarcoma. Of course, the gold standard for the diagnosis of cardiac masses remains histopathology. Other non-invasive imaging technologies such as cardiac CT and magnetic resonance imaging (MRI) have excellent diagnostic capability regarding tumour demarcation and spread.14,15 Likewise, RT3DE provides the spatial relationship with a tomographic perspective without the radiation or additional machines of CT or MRI requires.16,17 In certain instances, CT and MRI may be contraindicated, and they are generally much more costly than ultrasound imaging. Contraindications to the use of perflutren type compounds have recently been highlighted and should always be born in mind. Primary cardiac angiosarcoma most commonly arises in the right atrium, and frequently extends to the pericardium, vena cava, or tricuspid valve. In this case, there was no gross invasion into the vena cava or tricuspid valve, but there was involvement of the pericardium. Primary pericardial angiosarcoma can also present as a mass in continuity with the right atrial or right ventricular wall, even though it mainly originates from the right atrium. This case is the first report using perfusion imaging and RT3DE in a patient with primary cardiac angiosarcoma to define the tumour, inner morphology, and outer spatial characteristics with adjacent structures and then confirming the echocardiographic diagnosis with histopathology. With more experience and a systematized approach using cardiac ultrasound modalities with perfusion imaging, greater confidence can be attained in making a diagnosis for cardiac masses.
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Conclusions
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Given the rarity of this fatal condition and the lack of a specific
clinical presentation, the diagnosis of cardiac angiosarcoma
requires a high degree of suspicion. Complete echocardiography
including both contrast and RT3D may facilitate its diagnosis
in routine clinical practice. Early diagnosis remains elusive
and impacts prognosis. Novel ultrasound and perfusion tools
can be used to characterize the nature of a mass better.
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Supplementary data
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Supplementary data is available at European Journal of Echocardiography online.
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Abstract
Introduction
