Copyright © 2005, The European Society of Cardiology
Contrast echocardiography as a useful additional diagnostic tool in evaluating a primary cardiac tumor
aDepartment of Cardiology, Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, 2300 RC (or otherwise: Albinusdreef 2, 2333 ZA), Leiden, The Netherlands
bDepartment of Infectious Diseases, Leiden University Medical Center, The Netherlands
cDepartment of Radiology, Leiden University Medical Center, The Netherlands
dErasmus Medical Center, Rotterdam, The Netherlands
Received 27 September 2004; accepted after revision 6 February 2005.
m.c.p.haverkamp{at}lumc.nl
* Corresponding author. Tel.: +31 71 5262020; fax: +31 71 5266809.
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Abstract |
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The current report describes a case of a primary cardiac lymphoma. For early and appropriate treatment of a cardiac mass it is not only important to determine its localization and extension but also to differentiate between malignant and benign lesions. This report demonstrates that not only transthoracic echocardiography but also the other different forms of echocardiography such as transesophageal echocardiography, as well as contrast and intracardiac echocardiography, are useful tools in the diagnostic workup of cardiac masses.
Keywords: Cardiac mass; Echocardiography; Contrast echocardiography; Primary cardiac lymphoma; HIV
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Case report |
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A 48-year-old man was referred to our hospital for further diagnostic evaluation of a right cardiac process discovered at a transabdominal ultrasound study performed in the referring hospital. The patient had a 3 weeks history of recurrent fever (up to 40°C), night sweat and weight loss (9kg in 3 weeks). Except for general discomfort, the patient had no cardiac complaints.
On physical examination there was a regular and equal pulse of 88 beats per minute with a blood pressure of 110/70mmHg. Central venous pressure was normal. Auscultation of the heart and lungs revealed no abnormalities. Although the right upper abdomen was slightly tender, the liver was not enlarged. No lymphadenopathy was present.
Laboratory analysis showed minimal abnormalities: an elevated ESR (120mm/h), a slight normocytic anemia (hemoglobin concentration 7.0mmol/L) and an elevated lactate dehydrogenase (748U/L; normal <450U/L). Chest X-ray was normal. The ECG showed a prolonged PR interval of 220ms as the only abnormality.
Abdominal ultrasound did not show abnormalities in the abdominal cavity, but a solid, irregular thickening of the free wall of the right ventricle and right atrium and some pericardial effusion was noted.
Subsequently, transthoracic echocardiography (TTE) demonstrated a thickened free wall of the right ventricle with a lobulated, large, enhancing epicardial mass invading the right atrium and extending around the aortic root. There was also global pericardial effusion (Fig. 1A and B).
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Contrast echocardiography was performed by intravenously administrating SonoVue® (Bracco Inc. Diagnostics; sulphur hexafluoride 8µL/mL) with a single or two injections of 2–3mL in the maximum dose of 5mL per examination (Vivid 7 scanner 1.7MHz, power=mechanical index 0.07). Contrast perfusion imaging showed partial perfusion of the cardiac mass (Fig. 1C and D). Transesophageal echocardiography (TEE) confirmed a large mass invading the right atrium and the free wall of the right ventricle, which extended to the aortic root. Multislice computed tomography (MSCT) of the thorax and abdomen confirmed the echocardiographic findings and ruled out lymphadenopathy and other masses (Fig. 2). A primary cardiac neoplastic process was suspected. With combined intracardiac echocardiography and fluoroscopic guidance, percutaneous intracardiac biopsy of the right atrium and right ventricle was performed (Fig. 3A).5
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Subsequent histopathological examination revealed the presence of a blastic lymphoid infiltrative tumor that was CD45, CD20 and BCL6 positive. Immunocytology suggested an intermediate/high grade B-cell non-Hodgkin lymphoma, most probably diffuse large-B-cell lymphoma. In situ hybridization for Epstein–Barr virus (EBV) RNA and immunostaining for human herpes virus 8 (HHV-8) were negative. Human immunodeficiency virus (HIV-1) infection was confirmed by ELISA and subsequent immunoblotting. HIV viral load was 25,400copies/mL and the CD4 count was 123cells/mm3, indicating severely impaired cellular immunity.
Chemotherapy treatment consisting of doxorubicin, vincristine, cyclophosphamide and prednisone was initiated. During the first course of chemotherapy the atrioventricular conduction worsened with a prolonged PR interval and finally, the ECG showed a third degree atrioventricular block. Due to the absence of hemodynamic consequences, management was conservative.
After 3 months of treatment with chemotherapy the symptoms resolved and follow-up TTE showed an almost complete resolution of the cardiac mass (Fig. 3 B–D). The atrioventricular block resolved completely.
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Discussion |
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Lymphoma is the second most common tumor involving the heart in patients with the acquired immunodeficiency syndrome (AIDS).1 Cardiac involvement with non-Hodgkin lymphoma usually reflects systemic dissemination of the disease. Non-Hodgkin lymphomas in patients with AIDS usually derive from B-cells and are typically high grade and disseminated. However, even in AIDS patients, primary cardiac lymphoma is extremely rare.2–4 The lymphoma typically involves the right atrium and involvement of other chambers is less common.3,6 Most patients have no complaints or non-specific symptoms, but they can also present with pericardial effusion, heart failure and arrhythmias.2–4,7
Two-dimensional echocardiography is considered the most sensitive imaging modality for the evaluation of cardiac masses.7,8 Although a tissue diagnosis is still required, early differentiation between malignant and benign cardiac masses is important for early and appropriate treatment. Kirkpatrick et al. recently demonstrated the use of echocardiographic contrast perfusion imaging in differentiating the neo-vascularization of malignancies from the avascularity of thrombi or sparse vascularity of stromal tumors.9 The authors concluded that malignant and vascular tumors compared with adjacent myocardium showed hyper-enhancement following contrast administration.9 In the current patient, echocardiographic contrast perfusion imaging also showed hyper-enhancement of the tumor compared to adjacent myocardium, highly suspicious for malignancy. Subsequent TEE confirmed the extension of the tumor. Next myocardial biopsy was taken under the guidance of intracardiac echocardiography that allowed appropriate positioning of the biopsy catheter.5 Follow-up TTE during chemotherapy treatment showed a significant reduction of the tumor mass.
This report provides an example of how different forms of echocardiography (TTE, TEE, contrast echocardiography, intracardiac echocardiography) can be used to assist in a comprehensive evaluation of cardiac masses.
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Conclusion |
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Different echocardiographic techniques have been demonstrated to be useful in comprehensive evaluation of cardiac masses. In particular, contrast echocardiography to assess perfusion is a useful technique to further evaluate cardiac tumors. Early differentiation is important for further diagnosis, treatment and prognosis. Intracardiac echocardiography on the other hand, may be useful in guiding tissue biopsy.
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- Peterson C.D., Robinson Q.A., Kurnich J.E. Involvement of the heart and pericardium in malignant lymphoma. Am J Med Sci (1976) 272:161.[Web of Science][Medline]
- Roberts W.C. Primary and secondary neoplasm of the heart. Am J Cardiol (1997) 80:671–682.[CrossRef][Web of Science][Medline]
- Kaplan L.D., Afridi N.A., Holmvang G., Zukerberg L.R. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-2003. A 44-year-old man with HIV infection and a right atrial mass. N Engl J Med (2003) 349:1369–1377.
[Free Full Text] - Sanna P., Bertoni F., Zucca E., Roggero E., Passega Sidler E., Fiori G., et al. Cardiac involvement in HIV-related non-Hodgkin's lymphoma: a case report and short review of the literature. Ann Hematol (1998) 77:75–78.[CrossRef][Web of Science][Medline]
- Jongbloed M.R., Bax J.J., van der Burg A.E., Van der Wall E.E., Schalij M.J. Radiofrequency catheter ablation of ventricular tachycardia guided by intracardiac echocardiography. Eur J Echocardiogr (2004) 5:8–11.
[Free Full Text] - Araoz P.A., Ecklund H.E., Welch T.J., Breen J.F. CT and MR imaging of primary cardiac malignancies. Radiographics (1999) 19:1421–1434.
[Abstract/Free Full Text] - Meng Q., Lai H., Lima J., Tong W., Qian Y., Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol (2002) 84:69–75.[CrossRef][Web of Science][Medline]
- Tighe D.A., Anene C.A., Rousou J.A., King A.K., Engelman R.M. Primary cardiac lymphoma. Echocardiography (2000) 17:345–347.[CrossRef][Web of Science][Medline]
- Kirkpatrick J.S., Wong T., Bednarz J.E., Spencer K.T., Sugeng L., Ward R.P., et al. Differential diagnosis of cardiac masses using contrast echocardiographic perfusion imaging. J Am Coll Cardiol (2004) 43:1412–1419.
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