European Journal of Echocardiography 2008 9(1):101-102; doi:10.1016/j.euje.2007.03.040
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org.
Multicystic/cavitated giant left atrial myxomas: a matter of technology?
Juan Benezet-Mazuecos1,*,
Pedro Marcos-Alberca1,
Jeronimo Farre1,
Felix Manzarbeitia2,
Rosa Rabago1 and
Manuel Rey1
1 Cardiology Department, Laboratorio de Ecocardiografía, Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Avenida Reyes Católicos 2, 28040 Madrid, Spain
2 Pathology Department, Fundacion Jimenez Diaz, Universidad Autonoma de Madrid, Madrid, Spain
Received 22 February 2007; accepted after revision 24 March 2007; online publish-ahead-of-print 22 June 2007.
* Corresponding author. Tel: +34 91 550 48 80; fax: +34 91 549 70 33. E-mail address: jbenezet{at}yahoo.es
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Abstract
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We present the case of a rare echocardiographic image of a giant
cavitated myxoma and the pathologic findings of the cystic mass.
The new echocardiographic equipment not only has improved the
sensitivity for diagnosis of different pathologies but also
has redefined its visual and morphologic characteristics. Although
most myxomas are solid masses and some cystic myxomas have been
reported, the presence of multiple cavities on echocardiographic
exam has exceptionally been described. While cystic changes
have been described at autopsy in 14% of cardiac myxomas, its
identification with echocardiography is rare. Nowadays, the
new echocardiographic equipment has improved the quality and
the accuracy to detect and describe intracardiac masses, showing
myxomas with cystic cavities in vivo that in the past was a
pathologic finding.
Keywords: Atrial myxoma; Cystic mass; Echocardiography; Diagnosis
A 59-year-old woman, with no history of cardiac or systemic disorders, developed a slowly progressive history of dyspnea over the last 3 months. Physical examination was irrelevant except for the presence of basal rales and a diastolic murmur which was maximal at the lower left sternal border. The electrocardiogram showed a sinus rhythm with signs of left atrial (LA) enlargement. On the chest X-ray the heart had a normal size and there were pulmonary bilateral edema consistent with congestive heart failure. A large pedunculated mass in the LA was detected on an echocardiographic examination. Transesophageal echocardiography performed to define the mass confirmed the presence of a mobile non-homogenous mass measuring 55x30 mm and attached to the interatrial septum (Figure 1). This mass resulted in the obliteration of the LA cavity and prolapsed through the mitral valve into the left ventricle during diastole causing severe mitral valve obstruction. The giant mass was surgically removed and the postoperative course was uneventful. On macroscopic examination the resected tumor had a balloon like shape and a regular and smooth surface with cystic cavities inside filled with blood (Figure 2). The histological examination showed clustered multinucleated cells with the characteristic ovoid nucleus and eosinophilic cytoplasm (myxoma cells), and neovascular structures embedded in a myxoid stroma with cystic cavities and inflammatory cells. All these findings were compatible with the diagnosis of left atrial myxoma.
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Figure 1 (A) and (B) Transesophageal echocardiogram in horizontal (0º) and vertical (90º) views during systole showing the left atrium almost completely occupied by a huge non-homogenous mass with cystic cavities. The mass is attached to the mid-portion of the interauricular septum, close to the fossa ovalis. (C) and (D) Horizontal and vertical views in diastole disclosing the prolapse of the mass into the inflow tract of the left ventricle. (E) and (F) Color Doppler examination in diastole. Flow is allowed to pass into the LV through the narrow outer orifice not occupied by the mass. Proximal acceleration and turbulent flow denote the severity of the obstruction at the mitral level, confirmed with spectral continuous Doppler examination.
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Figure 2 (A) Echocardiogram showing a detail of the partly cystic tumor mass. (B) Macroscopic aspect of the resected mass. Glistening cut surface of the myxoma showing diffuse brown areas of hemorrhage and grey-tan tumoral tissue with the cystic cavity observed on the echocardiogram. (C) (HE, 10x). Microscopic panoramic view of homogeneous pink-blue extracellular matrix and the cystic cavities near the surface of the tumor, both with recent hemorrhage. (D) (HE, 200x) Myxoma cells arranged in cords, with moderate atypia of the nuclei and small amount of cytoplasm, floating in the rich in proteoglycans extracellular matrix with a companion of a few dispersed inflammatory cells.
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Atrial myxomas are the most common benign primary tumor of the
heart and they may be a major cause of patient morbidity and
mortality.
1,2 Clinical manifestations depend on the size and
chamber in which it is located, and include heart failure, embolization,
rhythm disturbances, syncope and even sudden death. Myxomas
rarely remain asymptomatic, especially if they are large.
1,2 The usual site of attachment is in the septum, in the area of
the fossa ovalis. It is rare to find a myxoma attached at other
localizations and this raises suspicion of malignancy.
1 Diagnosis
may be difficult in asymptomatic patients although echocardiography
can easily detect cardiac tumors. Therefore, the most useful
diagnostic tool for primary cardiac tumors is echocardiography,
especially transesophageal echocardiography, which in almost
all cases can precisely locate the tumor and define its extent.
The echocardiographic appearance of cardiac tumors may accurately
predict tumor type, malignant or benign.
3 Cavitated intracardiac
masses include cystic myxomas, hydatid cysts, right atrial pseudocysts
from peritoneovenous shunts, and thrombus. While cystic changes
have been described at autopsy in 14% of cardiac myxomas, its
identification with echocardiography is rare.
4 Although most
myxomas are solid masses and some cystic myxomas have been reported,
the presence of multiple cavities on echocardiographic exam
has exceptionally been described.
5 Three-dimensional (3D) echocardiography
is a new technique that offers a complete evaluation of size
and volume of intracardiac masses, allowing for accurate measurements
in multiple planes.
6 Nowadays, the new echocardiographic equipment
has improved the quality and the accuracy to detect and describe
intracardiac masses, showing myxomas with cystic cavities in
vivo that in the past was a pathologic finding.
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Supplementary material
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Supplementary material can be found in the online version.
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References
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