From the Third (H.T., K.A., T.H., T. Nakai) and First (T. Nakayama, T.U.)
Departments of Internal Medicine, Fukui Medical School, and the Department of
Internal Medicine (E.O.), Izumigaoka Onsen Hospital, Fukui, Japan.
A 67-year-old woman was admitted to a
hospital because of the recent onset of general malaise. She had a
classic lilac-colored rash over her eyelids, the bridge of her nose,
her cheeks, elbows, and knees and weakness in the proximal limb
muscles. A diagnosis of dermatomyositis was confirmed by skeletal
muscle biopsy. She was started on a course of oral glucocorticoids.
Three months later, she complained of dyspnea. An echocardiogram
revealed a massive pericardial effusion with evidence of both right
atrial and ventricular collapse consistent with
cardiac tamponade. Pericardiocentesis yielded 1000 mL of exudative
bloody fluid with a lactate dehydrogenase value of 23 950 IU/L.
Cytology revealed cells believed to represent lymphoma.
The patient was referred to Fukui Medical School for further
investigation in August 1995. Transesophageal
echocardiography showed a dense thick mass in the
right atrioventricular groove extending into the right
ventricular free wall (Fig 1A
Footnotes
Reprint requests to Hiroshi Tada, MD, the Third Department of Internal Medicine, Fukui Medical School, 23 Shimoaizuki, Matsuoka-cho, Fukui 910-11, Japan.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC1-267, Houston, TX 77030.
© 1998 American Heart Association, Inc.
Images in Cardiovascular Medicine
Primary Cardiac B-Cell Lymphoma
). The right ventricular
wall and the left ventricular inferior wall
were thickened and exhibited high echogenicities (Fig 1B
). A small
pericardial effusion was also noted. MRI demonstrated a thick mass in
the right atrioventricular groove and the thickened
right ventricular and left ventricular
inferior walls, suggesting significant massive cardiac
involvement (Fig 2A
through 2C). The
brightness of these lesions and pericardium was increased in
T2-weighted images, and Gd-DTPA enhanced the lesions
heterogeneously (Fig 2D
). Contrast right atrial angiography
demonstrated a zonal filling defect at the right
atrioventricular groove, indicating cardiac involvement
in that region (Fig 3
). Full-body CT and
MRI showed no abnormal lymph node swelling. 67Ga
scintigraphy revealed significant abnormal uptake in the
heart (Fig 4A
). Pericardiocentesis was
performed a second time to establish a definite diagnosis. Cytological
study showed cells consistent with large-cell lymphoma (Fig 4B
). Surface markers were positive for cluster of differentiation (CD)
19, CD22, CD45, and HLA-DR (human leukocyte antigen-Drelated) sIgM
and s
, indicating that the cells were of B-cell origin. Thus, the
patient was diagnosed with primary cardiac B-cell lymphoma with massive
cardiac involvement. She is currently undergoing medical treatment with
chemotherapeutic agents.

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Figure 1. Transesophageal
echocardiographic images. A, Dense, thick mass is
visible in right atrioventricular groove with extension
into right ventricular free wall. B, Right
ventricular wall is thickened and exhibits high
echogenicity. A small pericardial effusion is present. RA indicates
right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle;
and Ao, ascending aorta.

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Figure 2. ECG-gated, T1-weighted MRIs. A, Transverse
image through middle of right atrium showing thick mass in right
atrioventricular groove. B, Saggital image showing
thickened right ventricular wall. C and D, Saggital images
differing from B. Brightness of these lesions and pericardium are
increased on T2-weighted images, and Gd-DTPA enhances them
heterogeneously (D). PA indicates pulmonary artery.
Other abbreviations as in Fig 1
.

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Figure 3. Right atrial angiogram in right anterior
oblique projection. Massive involvement in right
atrioventricular groove appears as zonal filling
defect. Abbreviations as in previous Figures.

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Figure 4. A, 67Ga scintigraphic image.
Intense 67Ga uptake is visible in heart. B, Pericardial
fluid obtained from patient's second pericardiocentesis, showing
malignant cells with features of large-cell type of malignant lymphoma
(magnification x1000).
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