Chonnam Med J.  2014 Apr;50(1):27-30. 10.4068/cmj.2014.50.1.27.

Diffuse Infiltrative Primary Cardiac Lymphoma with Delayed Extracardiac Involvement

• Jeoung Y
• Lee SR
• Kim H
• Kim JW
• Lee JJ
• Chung SY

• KMID: 1889760
• DOI: http://doi.org/10.4068/cmj.2014.50.1.27

Abstract

Primary cardiac lymphoma (PCL) is an extremely rare and fatal neoplasm of the heart. Traditionally, it is defined as lymphoma involving the heart or pericardium. PCL has a poor prognosis because of the diagnostic difficulty and its location. We present the case of a 48-year-old man who presented with pericardial effusion and diffuse cardiac wall thickening. We first suspected infiltrative heart disease. However, even after performing a biopsy, we could not establish an accurate diagnosis. After 20 months, primary cardiac diffuse large B cell lymphoma (DLBCL) was diagnosed by cervical lymph node biopsy. In this case, after chemotherapy, the DLBCL lesions, including cardiac wall thickening, improved. The treatment outcome suggests that the diagnosis was diffuse infiltrative PCL with delayed extracardiac involvement.

Keyword

Heart; Lymphoma; Diagnosis

MeSH Terms

Biopsy
Diagnosis
Drug Therapy
Heart
Heart Diseases
Humans
Lymph Nodes
Lymphoma*
Lymphoma, B-Cell
Middle Aged
Pericardial Effusion
Pericardium
Prognosis
Treatment Outcome

Figure

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  FIG. 1 (A) Diffuse cardiac wall thickening was noted on two-dimensional echocardiography (parasternal long axis view). Echocardiography shows a large amount of pericardial effusion and right and left ventricular wall thickening. (B) Diffuse late gadolinium enhancement was observed on the T1-weighted cardiac magnetic resonance imaging. No intracardiac or extracardiac mass was observed. (C) No mediastinal mass is observed in the cardiac magnetic resonance imaging.

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  FIG. 2 Chest computed tomography. (A) Newly developed infiltrative soft tissue mass in the anterior mediastinum (10 months after the first admission). (B) Massive cervical lymphadenopathy was detected on the right side of the neck (20 months after the first admission).

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  FIG. 3 Chest computed tomography. (A) Before R-CHOP chemotherapy. (B) After the second cycle of R-CHOP chemotherapy, lymph node size, pericardial effusion, pleural effusion, and myocardial infiltration were reduced. (C) After the sixth cycle of R-CHOP chemotherapy, previous lesions were improved but the cardiophrenic lymph node lesion remained.

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