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Ann Thorac Surg 1996;61:1000-1001
© 1996 The Society of Thoracic Surgeons

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Case Report

Primary Cardiac Lymphoma

Departments of Surgery and Internal Medicine, Case Western Reserve University School of Medicine and Mt. Sinai Medical Center, Cleveland, Ohio

Accepted for publication October 4, 1995.

	Abstract

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A case of primary cardiac non-Hodgkin's B cell lymphoma is described in a patient presenting with obstructive right heart failure. Unlike the majority of cases, in this case the tumor was diagnosed ante mortem. The patient's history combined with the aggressive use of noninvasive echocardiography are helpful in diagnosing this rare lesion.

	Introduction

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Primary cardiac lymphoma, an extranodal malignant lymphoma of any cell type involving only the heart and pericardium without dissemination, is an extremely rare lesion, with fewer than 20 cases reported in the world literature [1–4]. The majority of these cases have been diagnosed post mortem, with an increasing frequency seen in immunocompromised individuals since 1987 [5–7]. We report a nonimmunosuppressed patient with primary cardiac lymphoma who presented with acute obstructive right heart failure.

The patient, a 63-year-old man with a prior history of malignant melanoma, clinically free of recurrence since 1980, presented for evaluation of malaise, weight gain, and exertional dyspnea. Physical examination revealed marked right-sided heart failure with neck vein distention, ascites, and pedal edema. Liver function tests had elevated results, with a serum glutamic-oxaloacetic transaminase level of 109 IU/L, a serum glutamic-pyruvic transaminase level of 113 IU/L, and a prothrombin time of 15.7 seconds.

Transesophageal echocardiography demonstrated a 7.5-cm lobulated mass filling the right atrium. The mass prolapsed through the tricuspid valve, compressing the valve leaflets and producing an area of high-velocity flow into the right ventricle with a mean gradient of 15.0 mm Hg. An inferior vena cavogram confirmed the presence of a mass prolapsing into the inferior vena cava with extension inferiorly below the level of the hepatic veins (Fig 1). Cardiac catheterization was performed, revealing normal coronary anatomy with the exception of collateral vessels that were perfusing the tumor from the right coronary artery.

View larger version (118K): [in this window] [in a new window]   Fig 1. . Right atrial involvement by the tumor.

At sternotomy, the heart appeared extremely cyanotic with markedly diminished contractility. The right atrium was massively dilated with extensive tumor involvement. The patient was placed on bypass using a right-angled venous cannula placed in the superior vena cava and a second venous cannula threaded to the retrohepatic inferior vena cava from the right femoral vein. Under 28 minutes of hypothermic circulatory arrest the right atrium was opened and a tumor measuring 7 x 6 x 4.5 cm was found to originate from the right atrial free wall with extension through the tricuspid valve and into the inferior vena cava. The tumor with the attached right atrial wall and involved inferior vena cava was excised and reconstructed with a bovine pericardial patch to achieve adequate surgical margins.

Histologic evaluation of the mass revealed a non-Hodgkin's B cell lymphoma. Immunoperoxidase staining demonstrated positive tumor staining for common leukocyte antigen and also L26 antigen, which is specific for B cell lymphoma. The neoplasm comprised dense sheets of large cells with prominent nucleoli and mitotic figures that insinuated into the underlying myocardial fibers. The tumor stained negative for CD3 (T cells) and MAK 6, which is an epithelial cell marker. The margins of resection were free of tumor.

The patient initially did well postoperatively. On postoperative day 9 he underwent a barium swallow, which showed no evidence of a duodenal leak, and he was started on oral alimentation. On postoperative day 20 an acute abdomen developed, and the patient was found at laparotomy to have a recurrent duodenal perforation. Despite operative repair of the perforation and broad-spectrum antibiotic coverage the patient became septic; multisystem organ failure developed, and he died on postoperative day 30.

	Comment

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Cardiac involvement in malignant lymphomas is relatively common, with 20% of patients showing cardiac involvement at the time of death [8]. Primary cardiac lymphomas in nonimmunocompromised individuals are exceedingly rare. Before 1987 there were only 20 reported cases of primary cardiac lymphomas in the English-language literature. Since 1987 there has been a significant increase in the presence of this lesion associated with immunosuppression, both in individuals iatrogenically immunosuppressed for solid organ transplantation and in patients positive for human immunodeficiency virus or patients with acquired immunodeficiency syndrome [4–7].

Although all histologic types of lymphoma involve the heart, those of the diffuse large cell or immunoblastic B cell subtype predominate. Burkitt's lymphoma of the heart has been reported on only two occasions.

Most patients with a primary cardiac lymphoma have a clinical course acute in onset with a rapid demise. Early diagnosis may best be made by echocardiography. Transesophageal echocardiography allows for architectural delineation of (1) single or multiple chamber involvement, (2) the focal or diffuse nature of the disease, and (3) the need for or feasibility of surgical resection.

Combined chemotherapy and radiation therapy may be appropriate in early stages. Obstructive symptoms or congestive heart failure often suggest mechanical inflow/outflow compromise. Surgical exploration is markedly aided by hypothermic circulatory arrest, which allows for a bloodless field to examine the extent of tumor involvement in the vena cava. Resection and reconstruction, if possible, may then be undertaken, assuring a tumor-free margin.

	Footnotes

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Address reprint requests to Dr Botham, Department of Surgery, Mt. Sinai Medical Center, One Mt. Sinai Dr, Cleveland, OH 44106.

	References

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1. McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978:99–100.
2. Chou S, Arkles LB, Gill GD, et al. Primary lymphoma of the heart. A case report. Cancer 1983;52:744–7.[Medline]
3. Bolis S, Bregani ER, Rossini F, Schiavina, Pogliani EM. Atrial flutter followed by sick sinus syndrome as presenting symptoms of B-cell malignant non-Hodgkin lymphoma involving the heart. Haematologica 1993;78:332–4.[Medline]
4. Roller MB, Manoharan A, Lvoff R. Primary cardiac lymphoma. Acta Haematol 1991;85:47–8.[Medline]
5. Constantino A, West TE, Gupta M, Loghmanee F. Primary cardiac lymphoma in a patient with acquired immune deficiency syndrome. Cancer 1987;60:2801–5.[Medline]
6. Gaurner J, Brynes RK, Chan WC, Birdsong G, Hertzler G. Primary non-Hodgkin's lymphoma of the heart in two patients with acquired immunodeficiency syndrome. Arch Pathol Lab Med 1987;111:254–6.[Medline]
7. Rodenburg CJ, Kluin P, Maes A, Paul LC. Malignant lymphoma confined to the heart, 13 years after a cadaver kidney transplant [Letter]. N Engl J Med 1985;313:122.[Medline]
8. McDonnell PJ, Mann RB, Buckley BH. Involvement of the heart by malignant lymphoma: a clinicopathologic study. Cancer 1982;49:944–51.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Mark J. Botham Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Margolin, D. A. Articles by Botham, M. J. Search for Related Content PubMed PubMed Citation Articles by Margolin, D. A. Articles by Botham, M. J.