J Cardiovasc Ultrasound.  2009 Dec;17(4):141-144. 10.4250/jcu.2009.17.4.141.

Extensive Primary Cardiac Lymphoma Diagnosed by Percutaneous Endomyocardial Biopsy

• Chin JY
• Chung MH
• Kim JJ
• Lee JH
• Kim JH
• Maeng IH
• Jung SY
• Hwang HJ
• Lee JB
• Youn HJ

• KMID: 1473718
• DOI: http://doi.org/10.4250/jcu.2009.17.4.141

Abstract

Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is rare in immunocompetent patients and represents 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. The clinical behavior is aggressive and the early symptoms are cardiac failure, syncope, arrhythmia, or pericardial effusion. Although echocardiography, computed tomography (CT) scan, magnetic resonance image (MRI) are the mainly used imaging techniques to detect cardiac tumors, pathologic examination is always required to confirm the diagnosis. Diagnosis of PCL is difficult due to non-specific clinical manifestations and requires invasive approach to get histopathologic evidence. While surgery with systemic chemotherapy or in combination with irradiation has been attempted, the only effective treatment is chemotherapy. However, the prognosis remains poor. We report on a 42-year-old woman who is diagnosed histopathologically as PCL by cardiac catheterization assisted percutaneous endomyocardial biopsy and treated successfully by anthracycline based chemotherapy.

Keyword

Non-Hodgkin's lymphoma; Cardiac tamponade; Pericardial effusion

MeSH Terms

Adult
Arrhythmias, Cardiac
Biopsy
Cardiac Catheterization
Cardiac Catheters
Cardiac Tamponade
Echocardiography
Female
Heart
Heart Failure
Heart Neoplasms
Humans
Lymphoma
Lymphoma, Non-Hodgkin
Magnetic Resonance Spectroscopy
Pericardial Effusion
Pericardium
Prognosis
Syncope

Figure

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  Fig. 1 Chest radiography demonstrates marked cardiomegaly with globular shape of heart.

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  Fig. 2 A: Apical 4 chamber view demonstrates infiltrative tumor mass in RV apical cavity (white arrow). B: On apical 2 chamber view, protruding mass (white arrow) is observed in LV apex with moderate amount of pericardial effusion. C: Parasternal short axis view at the mid-ventricular level shows moderate amount of pericardial effusion (white arrow) and mass in posteroinferior LV myocardium. LV: left ventricle, RV: right ventricle.

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  Fig. 3 A: Computed tomography reveals intracardiac mass involving both ventricles (black arrow). B: Magnetic resonance imaging (MRI) demonstrates mass on LV base which extended from pericardium to RV and LV (white arrow). C: PET/CT revealing malignant tumor involving the LV, RV and pericardial region as well as intense FDG uptake at LA and left lower paratracheal region and both kidneys. LV: left ventricle, RV: right ventricle, PET/CT: positron emission tomography/computed tomography, FDG: fluorodeoxyglucose, LA: left atrium.

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  Fig. 4 High power view of the left ventricular endomyocardial tumor. The tumor cells are atypical lymphocytes regarded as lymphoma cells. Hematoxylin and eosin stain (×100).

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  Fig. 5 A: Parasternal short axis view at the mid-ventricular level shows no more observed tumor mass and pericardial effusion. B: On apical 4 chamber view, there is no infiltrating cardiac mass in LV and RV apex. LV: left ventricle, RV: right ventricle.

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