Primary cardiac T-cell lymphoma

Abstract

Primary cardiac lymphoma (PCL), defined as a lymphoma clinically mimicking cardiac disease, with the bulk of the tumor located intrapericardially, is extremely rare in immunocompetent patients. Clinical manifestations vary depending on sites of involvement in the heart and include chest pain, arrhythmias, pericardial effusion, and heart failure. Diagnosis is often difficult and may require invasive procedures; in some cases, diagnosis is not made until autopsy. Histologically, nearly all cases of PCL reported thus far have been of B-cell origin. In this report, we describe a case of PCL of T-cell origin in an adult immunocompetent patient, the second reported in the literature to the best of our knowledge, and provide a brief overview of the features of previously published PCL cases.

Primary cardiac lymphoma

“…Histologically, the majority of the cases are diffuse large B-cell lymphoma (Fig. 2) [16], although rare cases of T-cell [11,12], anaplastic [13,14], and plasmablastic [15] lymphomas have also been described. An association between primary cardiac CD30+ anaplastic B-cell lymphoma and EBV nucleic acid sequences positivity (in the malignant cells) has been described [14,17].…”

“…The right atrium is the chamber most frequently compromised [10][11][12], followed by involvement of the right ventricle, left ventricle, left atrium, and the intraventricular septum [12]. Rarely, this tumor can arise from the tricuspid valve ring and the tricuspid valve leaflet [22].…”

“…PCL has no peculiar clinical picture [4], as symptoms may vary according to the heart sites involved [11,14]. The most frequent cardiac clinical manifestations associated with PCL are pericardial effusion, heart failure, and AV-block [18].…”

Primary Cardiac Lymphoma in an Immunocompetent 71-Year-Old Man

“…To our knowledge, only 23 cases of PCL in immunocompetent patients have been reported to date (Table I). [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] According to these reports, no patient presented with noncardiac lymphomatous localization, but, as in our patient, it was impossible to absolutely exclude noncardiac involvement in the absence of autopsic investiga- Nine of these patients were treated with surgical excision followed by chemotherapy, and 5 of them survived at least 6 months after treatment. No evidence exists concerning the necessity of surgery as an adjunct to chemotherapy in PCL; moreover, in those 9 cases surgery was the final diagnostic step, along with excision as treatment.…”

Sequential therapy of primary cardiac lymphoma with cardiectomy, total artificial heart support, and cardiac transplantation

“…4 The other 2 adult male patients with PCL of T-cell origin died within 1 month after diagnosis because of malignant arrhythmias. 5,6 Diagnoses were based on echocardiography, magnetic resonance imaging (MRI) and computed tomography (CT) to distinguish between PCL and cardiac involvement in generalized lymphoma. 7 Cytology of pericardial or pleural effusions has been diagnostic in only two thirds of samples, whereas endomyocardial biopsy has failed to establish diagnosis in half of the cases.…”