(Circulation. 1999;99:2342-2344.)
© 1999 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Primary Cardiac Malignancy Masquerading as Mitral Valve Stenosis
From the Divisions of Cardiology (V.D.S., R.G.S., M.A.) and Cardiothoracic Surgery (V.R.C.) and Department of Pathology (E.E.), University of Texas Medical Branch at Galveston.
A60-year-old white woman presented with a history of exertional shortness of breath rapidly progressing to orthopnea and episodes of paroxysmal nocturnal dyspnea over a period of 4 weeks. Her past medical history included hypertension; sick sinus syndrome, for which she had received a pacemaker; and a left carotid endarterectomy. Physical examination revealed normal vital signs, jugular venous distension of 5 cm, bibasilar rales, and a II/VI middiastolic murmur heard at the apex of the heart without an opening snap.
A transthoracic echocardiogram revealed a markedly
thickened anterior leaflet of the mitral valve with limited
diastolic excursion (Figure 1A
). A possible mass hugging the atrial
side of the anterior leaflet could not be excluded. The posterior
leaflet had preserved motion. Doppler
echocardiography across the mitral valve obtained a
mean gradient of 20 mm Hg, with a peak gradient of 47 mm Hg
and a calculated valve area of 1.57 cm2 by the
pressure half-time method (Figure 1B). The
transesophageal echocardiogram delineated a mass
involving the anterior leaflet of the mitral valve and extending to the
interatrial septum, with consequent obstruction of the valve (Figure 1C and 1D). The left atrium was otherwise normal.
|
The patient was treated symptomatically for
pulmonary edema, and after a coronary angiogram, which
revealed single-vessel disease, she underwent exploratory cardiac
surgery. During surgery, a large, multilobar, myxomatous mass 4 cm in
diameter was seen on the anterior leaflet of the mitral valve. The mass
had no distinct stalk, and it extended into the posterior commissure
and the interatrial septum (Figure 2A).
In addition, islands of fibrinous, gelatinous material that were
histologically similar to the mass were also visualized
in the left atrium. The patient underwent excision of the mass, mitral
valve replacement with a 27-mm St Jude's valve, and a right
coronary artery bypass graft.
|
Histologically, the mass consisted of a malignant
proliferation of spindle cells arranged in storiform pattern with focal
myxoid changes. The cells displayed marked nuclear pleomorphism with
occasional bizarre cells and frequent mitotic figures, including
abnormal forms (Figure 2B
and 2C). Immunohistochemistry revealed
negative staining for desmin and smooth muscle actin. Tumor cells were
strongly immunoreactive for CD-68 (Figure 2D) and focally with
desmin. Electron microscopy demonstrated features consistent
with a fibrohistocytic differentiation (Figure 2E). The
diagnosis was primary endocardiac malignant fibrous histiocytoma. The
patient underwent adjuvant chemotherapy and is alive and well 1 year
after surgery.
Footnotes
Reprint requests to R. Sheahan, MD, Division of Cardiology, University of Texas Medical Branch at Galveston, 301 University Blvd, Galveston, TX 77555.
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.
This article has been cited by other articles:
 |
|
 |
|
  G. Shanmugam Primary cardiac sarcoma. Eur. J. Cardiothorac. Surg., June 1, 2006; 29(6): 925 - 932. [Abstract] [Full Text] [PDF]
|
|
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||