European Journal of Echocardiography 2008 9(1):123-125; doi:10.1016/j.euje.2007.04.004
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Complete resection of a leiomyosarcoma of the left atrium invading the mitral anterior leaflet and obstructing the mitral orifice
Erdem Türky
lmaz1,
Fatih Ylmaz1,
Alper Özkan1,
Nur
en Kele1,
Mustafa Sa
lam1,
Osman Karakaya1,
Cevat Yakut2 and
Cihangir Kaymaz1,*
1 Kouyolu Heart and Research Hospital, Cardiology Clinic, 34846 Kartal, Istanbul, Turkey
2 Kouyolu Heart and Research Hospital, Cardiovascular Surgery Clinic, 34846 Kartal, Istanbul, Turkey
Received 7 January 2007; accepted after revision 6 April 2007; online publish-ahead-of-print 9 July 2007.
* Corresponding author. Tel: +90 0216 4594041; fax: +90 0216 4596321. E-mail address: cihangirkaymaz2002{at}yahoo.com
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Abstract
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Cardiac leiomyosarcomas are rare and highly invasive malignant
tumors. We report a 29-year-old female with mitral stenosis
symptomatology due to a left atrial leiomyosarcoma invading
mitral anterior leaflet.
Keywords: Cardiac leiomyosarcoma; Left ventricular inflow obstruction; Mitral stenosis
Cardiac leiomyosarcoma is a rare and highly invasive malignant
tumor associated with poor prognosis. The mitral valve is an
unusual location for leiomyosarcoma. We report a 29-year-old
female with mitral stenosis symptomatology due to a leiomyosarcoma
originating from the left atrium and invading the mitral anterior
leaflet. Initial assessment revealed an apical diastolic rumbling
murmur. Transthoracic and transesophageal echocardiography revealed
severe mitral orifice narrowing due to diffuse infiltration
of the anterior mitral leaflet by the tumor and an extension
of the tumoral mass into the whole surface of the left atrium
(
Figures 1 and
2A and
B). The mitral valve area was 1.3
cm
2 (by PHT method) and the calculated maximal and mean gradients
were 25 and 17 mmHg, respectively. Whole body CT scanning revealed
neither a probable extracardiac source nor a metastasis. The
tumor was completely resected (
Figure 3), and mitral valve
was replaced with a mechanical valve. Histopathological examination
of the tumor showed a low differentiated leiomyosarcoma (
Figure 4A and
B). The patient was discharged from hospital without complications
but refused further treatment. After four months, the clinical
and echocardiographic examination of the patient showed no signs
of recurrence or metastasis. She died suddenly at home 8 months
after surgery.
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Figure 1 Transthoracic echocardiography showing the thickened and rigid mitral anterior leaflet infiltrated by a large mass extending from left atrium wall into the anterior mitral leaflet (black arrow). Ao, Aorta; LA, left atrium; LV, left ventricle; and RV, right ventricle.
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Figure 2 Transesophageal echocardiography showing the tumor infiltrating the mitral anterior leaflet (A) and extending from the body of the mitral anterior leaflet into nearly the whole surface of the left atrium and appendage (white arrow) (B). AML, anterior mitral leaflet; other abbrevations are same as in Figure 1.
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Figure 3 Resected specimen including the partially infiltrated mitral valve apparatus and left atrial component of the tumor. Note the significant narrowing in the mitral valve orifice due to abnormal thickening of the mitral anterior leaflet by infiltration (black arrow).
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Figure 4 Histopathologic assessment of the specimen revealed fasiculated and cross breeding mesenchimal spindle-shaped myofibroblasts, pleomorphic nuclei and high grade mitotic figures inidicating the malignancy (H&E stain, x100) (A) and tumor focus on the left atrial side of the anterior mitral leaflet (black arrows). Note that tumoral infiltration is absent on the ventricular side of the leaflet (H&E stain, x40) (B).
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Supplementary material
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Supplementary material associated with this article can be found in the online version.
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References
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- Murphy MC, Sweeney MS, Putnam JB Jr, et al. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac Surg (1990) 49:612–18.[Abstract]
- Ghyra AS, Santander CK, Alarcon EC, Mucientes FH, Carrillo H. Leiomyosarcoma of the pulmonary veins with extension to the left atrium. Ann Thorac Surg (1996) 61:1840–1.[Abstract/Free Full Text]
- Minardi G, Pulignano G, Sentinelli S, Narducci C, Giovanni M. Left atrial leiomyosarcoma: double occurrence and double recurrence–report of one case. J Am Soc Echocardiogr (1998) 11:1171–6.[CrossRef][Web of Science][Medline]
- Ogimoto A, Hamada M, Ohtsuka T, et al. Rapid progression of primary cardiac leiomyosarcoma with obstruction of the left ventricular outflow tract and mitral stenosis. Intern Med (2003) 42:827–30.[CrossRef][Web of Science][Medline]
- Gurbuz A, Yetkin U, Yilik L, Ozdemir T, Turk F. A case of leiomyosarcoma originating from pulmonary vein, occluding mitral inflow. Heart Lung (2003) 32:210–4.[CrossRef][Web of Science][Medline]
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