Circulation. 2000;101:581-583
(Circulation. 2000;101:581.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Uterine Intravenous Leiomyomatosis Extending Through the Inferior Vena Cava Into the Right Cardiac Cavities
L. Cea-Calvo, MD;
F. Lozano, MD;
M. Pombo, MD;
A. Serrano, MD;
E. Rodríguez, MD;
J. Porto, MD;
A. Pozuelo, MD;
C. González, MD
From the Department of Medicine (L.C.-C., M.P., A.P., C.G.), Radiology
(F.L.), Pathology (A.S.), Heart Surgery (E.R.), and Vascular Surgery (J.P.),
Hospital Universitario Doce de Octubre, Madrid, Spain.
Correspondence to Dr L. Cea-Calvo, Hospital Universitario Doce de Octubre, Madrid, Departamento de Medicina Interna, Planta 13, Carretera de Andalucía, Km. 5,400, 28041 Madrid, Spain.
A41-year-old
woman was admitted to our hospital with a 3-week history of swollen
legs and abdominal distension that persisted after diuretic
therapy. Physical examination revealed a blood pressure of 110/70
mm Hg, a heart rate of 76 bpm, and a respiratory rate of 15 breaths
per minute. Cardiac auscultation was remarkable for a tricuspid
pansystolic regurgitation murmur. On abdominal
examination, a nontender mass resembling a uterus 12 to 13 weeks
pregnant was noted in the lower abdomen. An ECG showed sinus rhythm
with nonspecific findings, and the chest radiograph was normal. An
abdominal ultrasound revealed an elongated inferior vena
cava (IVC) and a filling defect inside the vein suggesting thrombosis.
A CT scan revealed a large, heterogeneous, and irregular
pelvic mass arising from the uterus (Figure 1
) and a thrombus-like image extending
from the IVC into the left renal vein (Figure 2) and up the right atrium.
Echocardiography showed a mobile mass extending
from the IVC through the right atrium and right ventricle (Figure 3), with its apparent tip moving within
the pulmonary valve, producing tricuspid
regurgitation.
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Figure 1. Axial pelvic CT: Well-delimited enhanced tumor,
displacing organs and underlying structures without infiltrating them,
with heterogeneous content arising from uterus.
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Figure 2. Axial contrast-enhanced abdominal CT showing a
widened IVC in its upper portion and a widened left renal vein with a
mass occupying vessel lumen, suggesting solid tumor and/or
thrombus.
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Figure 3. 2D echocardiogram revealing a mobile solid mass
extending into right atrium and ventricle, producing tricuspid
regurgitation. VD indicates right ventricle; AD, right
atrium; VI, left ventricle; AI, left atrium.
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A presumptive diagnosis of uterine intravenous
leiomyomatosis was made. MRI demonstrated a large mass in the uterus
extending via the left iliac vein and the inferior vena
cava into the right cardiac cavities, with the same signal intensity
through the full extent of the tumor (Figure 4). Angiography of the
inferior vena cava and iliac veins revealed almost complete
occlusion of the IVC, with prominent collateral circulation (Figure 5). Tumors in the heart and
inferior vena cava were successfully removed under deep
hypothermia and circulatory arrest (Figures 6 and 7).
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Figure 4. Coronal view on abdominal and pelvic MRI scan, at
retroperitoneal level, showing a cross section of abdominal vena cava.
Markedly dilated left iliac vein, left renal vein, and abdominal IVC,
with a solid cylindrical mass projecting into blood vessel lumens,
suggesting an intravenous continuous lesion.
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Figure 5. Ileocavography showing almost total occlusion of
primitive iliac veins and IVC, with collateral circulation.
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Figure 6. Specimen removed. Left, Intravenous
leiomyomatosis extending into right side of heart acquiring appearance
of IVC and right cavities. Right, Deformed uterus because of
proliferation of well-differentiated smooth muscle cells extending
through uterine veins.
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Figure 7. Intravascular mass, acquiring shape of vascular
bed, composed of mature muscular cells in a dense fibrous stroma, with
edematous areas occupying vessel lumen. Histological
study confirmed finding of intravenous
leiomyomatosis.
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Footnotes
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 A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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