Cardiothoracic Surgery: Cardiology and Cardiothoracic Surgery |

Primary Cardiac Lymphoma: A Rare Cause of Pericardial Tamponade FREE TO VIEW

Linh Truong, MD; Ching-Fei Chang, MD
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University of Southern California-LAC+USC, Los Angeles, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A52. doi:10.1016/j.chest.2016.02.055
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SESSION TITLE: Cardiology and Cardiothoracic Surgery

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Among myocardial malignancies, primary cardiac lymphoma (PCL) is very rare, with an incidence of only 1.3% and an estimated 197 case reports in the literature.1 We present a patient with PCL presenting with a pericardial effusion and early tamponade physiology.

CASE PRESENTATION: A previously healthy 52 year-old man presents with a 3-day history of left chest pain, dyspnea, and bilateral leg edema. Initial vitals and labs were unremarkable except for mild anemia (11.7 g/dL), elevated D-dimer (2108 ng/mL) and elevated LDH (412 U/L). A chest xray was normal except for an enlarged cardiac silhouette. Echocardiogram demonstrated a large pericardial effusion with early diastolic collapse of the right ventricle. A pericardiocentesis was performed which revealed a bloody lymphocytic-predominant effusion (97%) which was negative for atypical organisms but positive for cells consistent with diffuse large-B-cell lymphoma (DLBCL). PET-CT revealed an extensive cardiac mass infiltrating the septum, right atrium and ventricle, without evidence of extrathoracic mets. A diagnosis of primary cardiac lymphoma was made, and the patient underwent chemotherapy (R-EPOCH) with resolution of his disease on followup scanning.

DISCUSSION: PCL is defined as a Non-Hodgkin’s Lymphoma, most commonly DLBCL, which originates in the lymphatics around the heart and pericardium, and does not involve any other organ.2 It represents only 0.5% of all forms of extranodal lymphoma.1 Although extremely rare in immunocompetent patients, reports of PCL in patients with HIV and solid organ transplantation is on the rise. The mean age of presentation is 63 and there is a male:female predominance of 1.94. Most patients present with signs of arrhythmias, heart failure, tamponade, or syncope due to a myxoma effect.2 Over 90% originate in the right chambers, 41% involve the atrial septa, and 80% present with pericardial disease. PCL is extremely sensitive to chemotherapy, but unfortunately the treatment may cause sudden-death if there is rapid necrosis of the tumor leading to cardiac wall rupture. Our patient was very fortunate in regards to being diagnosed early, with only echocardiographic tamponade, and underwent chemotherapy without complications.

CONCLUSIONS: PCL is a very rare form of cardiac cancer that comprises only 1.3% of all cases, but may be increasing in incidence in the immunosuppressed population. The prognosis is usually poor. Clinicans should be aware of the classic features of this disease as demonstrated by our patient, since early recognition and treatment can lead to improve outcomes.

Reference #1: Petrich et al. Cancer. 2011;117(3):581-9

Reference #2: Miguel et al. Int J Cardiol. 2011;149(3):358-63

DISCLOSURE: The following authors have nothing to disclose: Linh Truong, Ching-Fei Chang

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