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Journal of Clinical Oncology, Vol 18, Issue 9 (May), 2000: 1998-1999
© 2000 American Society for Clinical Oncology


DIAGNOSIS IN ONCOLOGY

Non-Hodgkin's Lymphoma Involving the Heart

Arthur Skarin, MDConsultant Editor

Raymond Liang, C. M. Yu, W. Y. Au, C. K. W. Choy, Y. L. Kwong

Queen Mary Hospital, University of Hong Kong, Hong Kong

CASE 2. SECONDARY LYMPHOMA OF THE HEART MANIFESTING AS INTRACAVITARY MASSES

A 62-year-old retired civil servant presented with back pain for 5 months. Physical examination revealed no abnormality except mild tenderness over the upper thoracic spine. A magnetic resonance image of the thoracic spine showed a large irregular right para-spinal mass extending from T3 to T10 level and involving the vertebral bodies of T4 to T7 (Fig 1, arrows). The mass infiltrated the descending aorta anteriorly and compressed the spinal cord from T5 to T7 posteriorly. There was also involvement of the spinal muscle, ribs, adjacent pleura, and lung parenchyma. An open incisional biopsy was performed, which revealed a diffuse large B-cell lymphoma (Fig 2). Further staging investigations showed extensive involvement of the para-aortic lymph nodes. The bone marrow was not involved. He received chemotherapy consisting of methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, and dexamethasone.1 Local radiotherapy was also given. Unfortunately, only a partial remission could be achieved. Second-line chemotherapy was given and was followed by high-dose chemotherapy with autologous stem-cell rescue. This resulted in a clinical complete remission. The lymphoma, however, progressed a year later with extensive disease in the mediastinum and abdomen. A computed tomography scan of the thorax at that time showed a suspicious mass in the right atrium. Indeed, intracavitary masses compatible with lymphomatous infiltration were found in the two atria of the heart by two-dimensional transesophageal echocardiography (Fig 3, note sessile mass measuring 2.7 x 2.1 cm [arrows] in the right atrium attached to the interatrial septum; Fig 4, note a pedunculated mass measuring 1.2 x 1.5 cm in the left atrium [arrows] attached to the interatrial septum). Further chemotherapy and radiotherapy were given, but there was no significant clinical response. The patient eventually developed left hemiplegia, probably as the result of tumor embolization. As the terminal event, he succumbed to acute pulmonary edema that was likely the result of outflow obstruction by the tumor.



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Primary cardiac lymphoma is rare, but secondary involvement of the heart by lymphoma is not uncommon.2 The diagnosis is usually made late and the prognosis is often poor.2 Patients may have pericardial involvement or even cardiac tamponade. Myocardial involvement may also result in heart failure or arrhythmia. Lymphoma presenting as intracavitary lesions in the heart has been reported but is usually diagnosed at postmortem.3 Chest x-ray, transthoracic echocardiography, and computed tomography seem to have low sensitivity in detecting such a lesion.4-6 It has been reported that magnetic resonance imaging may have a higher sensitivity and may sometimes be helpful in these circumstances.6 On the other hand, this case has illustrated the clinical usefulness of transesophageal echocardiography in diagnosing lymphoma of the heart. Early detection and treatment may in some other cases prevent serious complications such as tumor embolization and outflow obstruction.

REFERENCES

1. Canellos GP, Skarin AT, Klatt MM, et al: The m-BACOD combination chemotherapy regimen in the treatment of diffuse large cell lymphoma. Semin Hematol 24:2-7, 1987

2. Chim CS, Chan ACL, Kwong YL, et al: Primary cardiac lymphoma. Am J Hematol 54:79-83, 1997[Medline]

3. Zyssman I, Cantor A, Steyn M, et al: Multiple intracavitary cardiac masses: An uncommon presentation of African Burkitt’s lymphoma. Int J Cardiol 37:421-423, 1992 [Medline]

4. Dorsay TA, Ho VB, Rovira MJ, et al: Primary cardiac lymphoma: CT and MR findings. J Comput Assist Tomogr 17:978-981, 1993[Medline]

5. Sanna P, Bertoni F, Zucca E, et al: Cardiac involvement in HIV-related non-Hodgkin’s lymphoma: A case report and short review of the literature. Ann Hematol 77:75-78, 1998[Medline]

6. Ceresoli GL, Ferreri AJ, Bucci E, et al: Primary cardiac lymphoma in immunocompetent patients: Diagnostic and therapeutic management. Cancer 80:1497-1506, 1997[Medline]




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