Primary cardiac non-Hodgkin’s lymphoma is defined as being exclusively located in the heart and/or pericardium, and is extremely rare. This disease occurs mainly in immunocompromised patients and rarely in the immunocompetent. To date, 35 cases of primary cardiac non-Hodgkin’s lymphoma have been reported in the literature by Chalabreysse et al in 2002, and 22 of these cases were diffuse, large B-cell lymphoma (DLBCL). We report a rare case of an immunocompetent female with no significant medical history who presented with dyspnea, chest pain and the beginnings of an SVC syndrome. The patient was initially diagnosed with primary cardiac Burkitt’s lymphoma when surgical pathology was reviewed. After further investigation by another pathology lab, the tumor was defined as DLBCL, which was confirmed by fluorescence in situ hybridization techniques. J INVASIVE CARDIOL 2008;20:E59–E60

Figure 2A.

(A) Computed tomographic (CT) scan with pulmonary embolism protocol revealing a large soft tissue mass centered in the right atrium measuring approximately 3 x 4.5 cm and extending into the inferior and superior vena cavae (arrow), causing a narrowing.

Figure 1.

Computed tomographic scan demonstrating a soft tissue mass extending around the right pulmonary artery (arrow) and inferior vena cava, causing a narrowing.

       Primary cardiac non-Hodgkin’s lymphoma is defined as being exclusively located in the heart and/or pericardium, and is extremely rare. This disease occurs mainly in immunocompromised patients and rarely in the immunocompetent. In a series of over 12,000 autopsies, only 7 primary tumors were identified for an incidence of less than 0.1%.¹ Primary cardiac lymphoma comprises only 2% of all cardiac tumors, and may obstruct valvular orifices and even cause hypertrophic cardiomyopathy when heavy tumorous lesions infiltrate the ventricular septum, as described by Roberts et al.² Metastatic involvement of the heart and pericardium is over 20 times more common and has been reported on autopsy series in up to 1 in 5 patients who died from cancer.^1,3–5
       To date, 35 cases of primary cardiac non-Hodgkin’s lymphoma have been reported in the literature by Chalabreysse et al in 2002, and 22 of these cases were diffuse, large B-cell lymphoma (DLBCL).⁶ We report a rare case of an immunocompetent female with no significant medical history who presented with dyspnea, chest pain and the beginnings of a superior vena cava (SVC) syndrome. The patient was initially diagnosed with primary cardiac Burkitt’s lymphoma when surgical pathology was reviewed. After further investigation by another pathology lab, the tumor was defined as DLBCL, which was confirmed by fluorescence in situ hybridization techniques.

       Case Report. A 74-year-old female was admitted to an outside hospital with severe shortness of breath and left upper extremity and facial swelling. Several months prior to admission, she had been experiencing dyspnea on exertion after her usual activities of playing intramural basketball. Also, she began to notice that after reclining all night, upon awakening in the morning, her head was swollen and red. This gradually subsided as the day progressed. Initially, the patient had undergone a computed tomographic (CT) scan with pulmonary embolism protocol revealing a large soft-tissue mass centered in the right atrium and measuring approximately 3 cm x 4.5 cm and extending into the inferior and SVC, causing a narrowing (Figures 2A and B). An adenosine Technetium-99m tetrofosmi stress test was done which showed no defects; this was followed by an echocardiogram revealing a right atrial mass measuring 2 cm x 2 cm without collapse. The patient was transferred to our center for cardiovascular surgical evaluation.

Figure 3.

Computed tomographic scan of the chest revealed a markedly narrowed superior vena cava with prominent collateral veins and shunting of blood into the azygos system (arrow).

Figure 2B.

(B) Analagous threedimensional CT scan.

       Additionally, transesophageal echocardiography (TEE) and a repeat computed tomographic (CT) scan of the chest revealed an evolving right atrial mass now measuring 3 cm x 4.7 cm, with a markedly narrowed SVC with prominent collateral veins and shunting of blood into the azygos system (Figure 3). The patient underwent surgery for debulking to relieve the SVC obstruction. Previous to her surgery, the patient underwent coronary angiography, which revealed no evidence of occlusive coronary artery disease.
       Surgical pathology revealed a high-grade B-cell lymphoma positive for CD20, CD79a and BCl-2. Hematology was consulted and performed a bone marrow biopsy without evidence of involvement by B-cell lymphoma. The patient was started on CytaBOM (cyclophosphamide, doxorubicin, etoposide, methotrexate with leucovorin, and prednisone-cytarabine, bleomycin, vincristine, and methotrexate with leucovorin) chemotherapy. Serologies for Epstein-Barr virus and human immunodeficiency virus (HIV) were negative.

       Discussion. Primary cardiac lymphoma is a rare disease and is almost unheard of in the immunocompetent patient. Recently, the incidence of lymphoma or atypical lymphoid proliferation associated with HIV or transplant recipients has increased.⁶ Primary cardiac lymphoma usually occurs in adults (mean age 62.1 years; 13–90 years), with a slight male predominance (22:13). Usually, the bone marrow is not involved and rarely is associated with lymphadenopathy.⁶
       DLBCL is one of the clinically aggressive non-Hodgkin’s lymphomas, generally having a high proliferation fraction (> 40%). DLBCL comprises 30% of all non-Hodgkin’s lymphomas, with an incidence of 2.9 cases/100,000 adults per year.⁷ Patients who are middle-aged or older usually present with symptoms of a large mass in the neck or abdominal region secondary to bulky lymphadenopathy. There is a rare variant of DLBCL which presents as primary mediastinal large B-cell lymphoma that is thymic in origin, with the most common clinical presentation being that of SVC syndrome/compromise.
       To date, only 35 cases of primary cardiac lymphoma have been reported in the English and French literature by Chalabreysse et al.⁶ Our patient had a primary cardiac lymphoma that was defined as DLBCL, with the presenting symptom of dyspnea on exertion. This case is truly unusual due to the patient’s prior good health and immune status. Primary cardiac lymphoma and other more common cardiac tumors are usually not detected until symptoms arise, which are secondary to obstruction, local invasion or embolization. Chest pain and dyspnea are the most common symptoms in 26% and 20% of patients, respectively, with SVC syndrome in 9%.^6,8–10 Valvular disruption causing regurgitation and arrythmias secondary to invasion of the conduction system may also arise less frequently. Two dimensional echocardiography is the most common modality of diagnosis, with the caveat that the upper part of the right atrium, pulmonary vessels and SVC may be difficult to analyze.⁹ TEE is superior to conventional two-dimensional echocardiography due to the fact that the patient’s body structure does not influence imaging and can give optimal images of the cardiac base.^9,11 CT offers better accuracy than TEE in the detection of cardiac/pericardial masses; 93% were detected in literature reviews.9 Magnetic resonance imaging gives 100% positive results in the literature.¹² Drainage of pericardial fluid may be both therapeutic and diagnostic.
       Prognosis for primary cardiac lymphoma is generally poor and late diagnosis is a major factor in outcome. Whatever the treatment, 60% of the patients reviewed died an average of 1.8 months after diagnosis.⁶ Primary cardiac lymphoma should be treated like other aggressive non-Hodgkin’s lymphomas arising elsewhere in the body, with multi-agent anthracyline-based chemotherapy and possible radiation therapy.