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Right Atrial Myxoma

Ramin Khorasani, MD
Jeffrey L Jorgensen, MD
Douglass F Adams, MD

September 10, 1994

Presentation

A 56-year-old woman presented with distended neck veins.

Imaging Findings

Axial T1 MRI
Axial Balanced MRI
Axial T1 gadolinium-balanced MRI
Coronal T1 MRI
Gross Pathology
Microscopic Pathology

Chest radiograph was normal. Cardiac evaluation included an echocardiogram, which demonstrated a right atrial mass possibly extending into the inferior vena cava (IVC).

The MRI examination included ECG-gated spin echo as well as cine sequences. T1-weighted coronal and axial images show a slightly lobular right atrial mass (arrows). This mass is of homogeneous intermediate signal intensity, heterogeneously enhanced with gadolinium (arrows), and measures 5 cm in largest dimension. The mass extends to the origin of the IVC but does not extend more inferiorly. On an axial balanced sequence (intermediate between T1 and T2), the mass extends into the right ventricle (arrows).

Differential Diagnosis

The presence of contrast enhancement in this mass makes the diagnosis of thrombus, the most common intracardiac mass, unlikely in this patient. A primary intracardiac tumor is the most likely possibility. The most frequent benign tumors are myxoma and lipoma, and the most ommon malignant tumor is angiosarcoma. Secondary tumors of the heart occur 40-50 times more frequently than primary cardiac tumors but are less likely in this patient because of the lack of a known primary neoplasm, absence of other metastatic foci, and lack of extension into the IVC as seen in renal cell carcinoma.

Diagnosis

Right atrial myxoma

Discussion

Radiology

Myxoma is the most common benign intracardiac tumor in adults accounting for nearly 40% of all cardiac tumors. Myxoma is three times more commonly seen in the left atrium compared to the right. A ventricular location is unusual. The tumor may protrude into a ventricle causing partial obstruction of the atrioventricular valve.

MRI has demonstrated the various configurations of the myxoma: [1] a spherical mass with a narrow pedicle attached to the interatrial septum, [2] a tumor with a wide base of septal attachment, or [3] a tumor attached to the atrial side of the mitral valve.

Pathology

Myxomas are the most common primary cardiac neoplasm, often arising from the intraatrial septum (left more often than right). As in this patient, the tumors are often pedunculated and/or lobulated gelatinous masses that can obstruct or swing back and forth through the atrioventricular valves, damaging the valve cusps.

Microscopically, myxomas have an abundant mucopolysaccharide matrix and vascular channels with varying numbers of distinctive stellate or plump "myxoma" cells (left slide). Tumor-associated thrombus, degeneration, hemorrhage, calcification, inflammatory infiltrate (basophils) or superinfection may lead to an incorrect diagnosis of mural thrombi or endocardial vegetations (right slide). Similar ardiac presentation of sarcomas have been rarely described and may present initial diagnostic confusion.

References

1. Higgins CB, Hricak H, Helms CA. Magnetic resonance imaging of the body. 2nd ed. New York: Raven, 1992: 551-558.

2. Freedberg RS, Kronzon I, Rumanik WM, Liebeskind D. The ontribution of MRI to the evaluation of intracardiac tumors diagnosed by echocardiography. Circulation 1988;77:94-103.


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