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Diffuse Cardiac Sarcoma Mimicking Myocardial Hypertrophy

Michel Zuber1, Jan-Olaf Gebbers3, Peter Buser4, Verena Briner2, Paul Erne1
1Division of Cardiology, 2Department of Medicine, 3Institute of Pathology, 4Kantonsspital Luzern and Department of Cardiology, University Hospital Basel, Switzerland

A 69-year-old woman was referred for hypertensive cardiomyopathy with ventricular tachycardia and right-bundle-branch-block morphology. She had diagnosed her own arterial hypertension by measuring blood pressure and was taking the same antihypertensive drugs as her sister did for ten years. For the last 6 months she noticed angina pectoris Canadian Cardiovascular Society (CCS) grade II, shortness of breath, and general fatigue. We found a blood pressure of 160/106 mm Hg. The ESR was 25 mm/h, creatine kinase was not elevated, but the troponin T test was abnormal. Echocardiography showed a small pericardial effusion, concentric hypertrophic myocardium with irregular structures, and global systolic dysfunction with reduced ejection fraction of 32% (Fig. 1A). Diffuse myocardial thickening was seen in MRI (Fig. 1B). Doppler sonography was indicative of restrictive physiology. Coronary angiography revealed normal coronary and renal arteries and in the electrophysiologic examination, complex ventricular arrhythmias could be induced from both ventricles. The patient died within one month due to untreatable repetitive pulmonary oedema. Only the postmortem study revealed the rare finding of diffuse myocardial infiltration of a cardiac sarcoma (heart 838 g) (Figs. 1C, 1D) with metastases to lungs, thyroid, liver, kidneys, adrenal gland, and skeleton.

Figure legend

Figure 1. A: Parasternal long-axis view with echocardiography. B: Cardiac MRI image in axial plane. C: Gross specimen with opened left ventricle and multiple nodules of a sarcoma (*) mimicking myocardial hypertrophy. D: Immunohistochemistry of smooth muscle actin in the undifferentiated cardiac sarcoma cells (red) infiltrating the myocardium (unstained). APAAP technique.



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