Primary cardiac lymphoma is rare, accounting for 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas.¹

Adults are typically affected with a mean age of ˜60 years at presentation (range, 12 to 86 years).² The presenting symptoms are most commonly related to congestive heart failure, pleural effusions, or conduction system disturbances. Anginal pain may indicate infiltration of a coronary artery.³

There have been occasional reports of cardiac lymphoma, generally associated with extracardiac disease, in patients with AIDS or solid organ transplant.^2,4 Cardiac lymphoma after orthotopic heart transplant is rare.

The sites of the heart most often affected by lymphoma are the right atrium, followed by the right ventricle, left ventricle, left atrium, atrial septum, and ventricular septum^2,5 (Table 185.1). More than one cardiac chamber is involved in over 75% of cases.

Primary cardiac lymphomas typically occur as multiple masses of firm, white, homogeneous nodules with the “fish flesh” consistency (Figs. 185.1 and 185.2). The heart is usually enlarged at autopsy or explant.^2,6 The gross appearance may suggest sarcoidosis. However, sarcoid granulomas do not usually form large nodules that extend onto epicardium/pericardium, as is typical for lymphoma, and are generally more firm and fibrous.

The most common types of lymphomas involving the heart are diffuse large B-cell lymphoma, followed by Burkitt lymphoma, follicular lymphoma, effusion lymphoma, anaplastic large cell lymphoma, and peripheral T-cell lymphoma (Table 185.1)2 (Figs. 185.3, 185.4, 185.5, 185.6).

Survival is generally less than a month without treatment, but survival up to 5 years has been documented.¹ Patients treated with the CHOP (cyclophosphamide, hydroxydaunorubicin/adriamycin, oncovin, prednisone) survive up to 18 months; those treated with the BACOP (bleomycin, doxorubicin, cyclophosphamide, vincristine, and prednisone) survive up to 49 months; patients who are treated surgically survive more than 18 months; and those who undergo radiation therapy survive for up to 15 months.⁶

Considered a histiocytosis in the juvenile xanthogranuloma group, Erdheim-Chester histiocytosis typically involves the eyes, lungs, retroperitoneum, and pituitary glands, with characteristic radiologic abnormalities of the long bones (see Chapter 92). Clinical cardiovascular involvement occurs in 5% to 10% (although this estimate may be low7) and is manifest as aortic, myocardial, or pericardial disease.⁸ The heart or aorta is rarely biopsied for diagnosis, which is generally made
by computed tomography or magnetic resonance imaging.⁹ By sensitive imaging study, 56% of patients with Erdheim-Chester disease have periaortic fibrosis, 44% pericardial involvement, and 31% myocardial involvement, often involving the right atrium.⁷