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Journal of Clinical Oncology, Vol 26, No 1 (January 1), 2008: pp. 150-152 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.12.5955
Primary Cardiac Lymphoma Mimicking Left Atrial Myxoma in an Immunocompetent PatientDepartment of Medicine, Division of Hematology/Oncology, University of Texas Health Science Center in San Antonio, San Antonio, TX
Department of Pathology, University of Texas Health Science Center in San Antonio, San Antonio, TX A 52-year-old obese Hispanic woman presented in March 2005 with a 1-week history of dizziness, leg swelling, orthopnea, exertional dyspnea, and nonradiating pain in the upper back. In the prior 2 months she also had paroxysmal cough and a 7-kg weight loss, but no fever, chills, or night sweats. She had type 2 diabetes, hypertension, and was status postangioplasty for renal artery aneurysm. She had bilateral jugular venous pulsation and basilar crackles, irregularly irregular rate and rhythm, 2 of 6 intensity systolic ejection murmur at the left sternal border, and trace edema of lower extremities. Notable laboratory values included hemoglobin of 9.3/dL, creatinine of 1.3 mg/dL, and erythrocyte sedimentation rate of 117 mm/h. Normal results included those for cardiac enzymes and HIV serology. Outside transthoracic echocardiography, confirmed after referral for resection, showed a left atrial mass of 7 x 3 cm that prolapsed intermittently through the mitral valve causing mitral regurgitation (Fig 1). Left ventricular systolic function was adequate. Chest x-ray showed cardiomegaly and mild pulmonary edema. ECG showed atrial fibrillation and lateral lead ST depression. Cardiac catheterization showed coronary artery stenoses up to 90% (of left anterior descending artery) and tumor blood supply from an atrial branch of the circumflex artery. Preoperative diagnosis was left atrial myxoma. Intraoperatively, the tumor was found friable, widely attached to the atrial septum and to the roof of the left atrium laterally to the superior vena cava juncture. A smaller tumor was between trabeculae and in the right lower pulmonary vein. Both were resected, preceded by two-vessel coronary artery bypass graft. The resected mass consisted of several fragments of soft tan-brown tissue (the largest one was 7 x 6 x 3.5 cm), together measuring up to 8 cm (Fig 2). On microscopy, fringes of tumor infiltrated adjacent cardiac muscle and other portions were necrotic. Tumor cells were intermediate to large transformed (noncleaved) lymphocytes, many exhibiting a plasmacytoid morphology with round nuclei and eosinophilic cytoplasm; other cells had nuclei with folded or irregular borders (Fig 2). On paraffin immunoperoxidase stains, tumor cells were CD20+ coexpressing CD5, BCL-6, BCL-2, and up to 90% MIB-1 (Ki67). They were negative for CD10, EBER, CD23, cyclin D1, HHV8, CD43, CD2, CD138, CD56, immunoglobulin G (IgG), IgA, IgM, and light chains. The final diagnosis was diffuse large B-cell lymphoma. The postoperative course was complicated by cardiac and renal failure requiring transient ventilatory support, and hemodialysis. Obtundation and divergent gaze on day 13 was explained by magnetic resonance imaging, showing multiple small embolic infarctions. CSF cell count was normal and cytology was negative for malignancy. The patient received anticoagulants, recovered neurologically, and spontaneously converted to sinus rhythm on postoperative day 39. Computed tomography imaging showed lymph nodes of up to 1.5 cm in the aortocaval, right iliac, and left inguinal area. Bilateral bone marrow biopsies and aspirate were negative, including cytogenetic and flow cytometry immunophenotyping studies. On day 30 after surgery, cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab chemotherapy was started. After five cycles, it was terminated because of symptoms of undue fatigue. Work-up revealed occlusion of the internal mammary bypass graft to the left anterior descending artery. Transthoracic echocardiography was negative. Computed tomography restaging showed remission of subdiaphragmal lymphadenopathy. Reimaging in March 2007 confirmed complete remission. The patient is currently well 26 months after resection, and 23 months after last chemotherapy.
Primary tumors of the heart are rare and 75% are benign. About half of these are solitary myxomas, usually involving the left atrium.1 Disseminated cancers and lymphomas rarely involve the heart, and usually have no cardiac symptoms.2 In contrast, primary cardiac non-Hodgkin's lymphomas (PCLs) involve heart and/or pericardium exclusively3 or most prominently.4,5 They are extremely rare, accounting for 1% to 2% of primary cardiac tumors in immunocompetent patients, and involve the right versus the left atrium at a ratio of about 8:1.4-12 Most reports concern single or few cases, some with reviews.5-8 HIV infection or post-transplantation immunosuppression seem to increase risk.4,13 PCLs (as any cardiac tumor) present according to site and extent of disease, with any combination of constitutional symptoms, chest pain, heart failure, sudden arrhythmia death, pericardial effusion/tamponade, superior vena cava syndrome, valvular disease, mass effect/invasion, or pulmonary or systemic embolism. Protean presentation, rapid progression, and rarity are challenges for timely diagnosis of PCL. Postmortem diagnosis was the rule before the advent of echocardiography.4 Open biopsy scored the highest sensitivity in one review,5 but transthoracic or transvenous biopsy/fine-needle cytology led to diagnosis in more recent reports.8,9 PCLs typically are invasive large B-cell lymphoma,4,6,8 of a CD5+ cell type noted for extranodular and aggressive growth.14 Our case fits these traits: myocardial invasion, tumor cells positive for CD20 and CD5, 90% expressing MIB-1, but cyclin D1 negative. For treatment, resection of PCLs alone has resulted in survival of up to 72 months or more in rare cases.15 With use of effective systemic treatment, gross resection seems unnecessary,5,8 but relapses were seen up to 12 months after chemotherapy.8 Recent patients have experienced more than 36 months of remission after adjuvant16 or primary chemotherapy.9,10,17,18 Additional radiation does not improve chemotherapy results.8 There is often no alternative for chemotherapy in PCLs, but several early deaths have been described (eg, due to arrhythmia, thrombosis, or neutropenic fever).7,8,12 In summary, this immunocompetent patient presented with a symptomatic left atrial lesion, which after resection as presumed myxoma, was diagnosed as invasive cardiac diffuse large B-cell lymphoma, with some lymphonodular involvement. She lives in complete remission, 26+ months after surgery, and 23+ months after finishing cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab chemotherapy. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
ACKNOWLEDGMENTS Presented at Annual Research Medicine Day, May 23, 2006, University of Texas Health Science Center in San Antonio, San Antonio, TX. REFERENCES
1. Reynen K: Cardiac myxomas. N Engl J Med 333:1610-1617, 1995 2. McDonnell PJ, Mann RB, Bulkley BH: Involvement of the heart by malignant lymphoma: A clinicopathologic study. Cancer 49:944-951, 1982[CrossRef][Medline] 3. McAllister HA Jr, Hall RJ, Cooley DA: Tumors of the heart and pericardium. Curr Probl Cardiol 24:57-116, 1999[Medline] 4. Burke A, Virmani R: Tumors of the Heart and Great Vessels. Washington, DC, Armed Forces Institute of Pathology, 1995 5. Ceresoli GL, Ferreri AJ, Bucci E, et al: Primary cardiac lymphoma in immunocompetent patients: Diagnostic and therapeutic management. Cancer 80:1497-1506, 1997[CrossRef][Medline] 6. Chalabreysse L, Berger F, Loire R, et al: Primary cardiac lymphoma in immunocompetent patients: A report of three cases and review of the literature. Virchows Arch 441:456-461, 2002[CrossRef][Medline] 7. Rolla G, Bertero MT, Pastena G, et al: Primary lymphoma of the heart: A case report and review of the literature. Leuk Res 26:117-120, 2002[CrossRef][Medline] 8. Ikeda H, Nakamura S, Nishimaki H, et al: Primary lymphoma of the heart: Case report and literature review. Pathol Int 54:187-195, 2004[CrossRef][Medline] 9. Maier BO, von Scheidt W, Sciuk J, et al: Cardiac involvement in malignancies: Case 4. Primary cardiac diffuse large B-cell lymphoma—Diagnosis by transesophageal echocardiography-guided transvenous biopsy. J Clin Oncol 22:2745-2747, 2004 10. Thompson MA, Harker-Murray A, Locketz AJ, et al: Unusual lymphoma manifestations: Case 2. Myocardial lymphoma presenting as atrial flutter. J Clin Oncol 22:558-560, 2004 11. Rockwell L, Hetzel P, Freeman JK, et al: Cardiac involvement in malignancies: Case 3. Primary cardiac lymphoma. J Clin Oncol 22:2744-2745, 2004 12. Zakynthinos E, Tassopoulos G, Haritos C, et al: Huge biatrial primary cardiac B-cell lymphoma resulting in bilateral atrioventricular valve obstruction. Leuk Lymphoma 45:2339-2342, 2004[CrossRef][Medline] 13. Duong M, Dubois C, Buisson M, et al: Non-Hodgkin's lymphoma of the heart in patients infected with human immunodeficiency virus. Clin Cardiol 20:497-502, 1997[Medline] 14. Yamaguchi M, Seto M, Okamoto M, et al: De novo CD5+ diffuse large B-cell lymphoma: A clinicopathologic study of 109 patients. Blood 99:815-821, 2002 15. Serrano M, Iglesias A, Bellas C, et al: Left atrial ball thrombus with histologic features of extranodal B-cell lymphoma: Prolonged survival after surgery. Acta Oncol 33:575-576, 1994[Medline] 16. Quigley MM, Schwartzman E, Boswell PD, et al: A unique atrial primary cardiac lymphoma mimicking myxoma presenting with embolic stroke: A case report. Blood 101:4708-4710, 2003 17. Nand S, Mullen GM, Lonchyna VA, et al: Primary lymphoma of the heart: Prolonged survival with early systemic therapy in a patient. Cancer 68:2289-2292, 1991[CrossRef][Medline] 18. Yates A, Huber S, Unger B, et al: Survival for three years after intramyocardial high malignant non-Hodgkin lymphoma. Cardiovasc Surg 11:321-323, 2003[CrossRef][Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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