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Journal of Clinical Oncology, Vol 22, No 13 (July 1), 2004: pp. 2745-2747 © 2004 American Society of Clinical Oncology. DOI: 10.1200/JCO.2004.08.131
Cardiac Involvement in MalignanciesCASE 4. Primary Cardiac Diffuse Large B-Cell Lymphoma: Diagnosis by Transesophageal Echocardiography-Guided Transvenous BiopsyDepartments of Hematology and Oncology, Cardiology, and Nuclear Medicine, Institute of Pathology, Klinikum Augsburg, Augsburg, Germany A 63-year-old woman presented to the emergency department with recurring drop attacks which were related to a bradyarrhythmia absoluta. She had a history of a carcinoma of the left breast 15 years ago, treated by surgery and radiotherapy. The bradyarrhythmia was documented by ECG and correlated strongly with the severity of symptoms. She was admitted to a cardiac ward for cardioversion and pacemaker implantation. Before admission, a transthoracic echocardiography did not show any relevant morphological alterations. A transesophageal echocardiography (TEE) surprisingly revealed a signal intense mass at the atrial septum (Fig 1). Further examinations and computed tomography scan of the chest confirmed the finding of an intracardiac mass, which was morphologically suspicious of being malignant. It was situated at the ventricular septum and the right atrium. In addition to the cardiac involvement, the computed tomography scan showed some enlarged mediastinal lymph nodes. After implantation of a VVI-pacemaker, the patient was transferred to an oncology ward. Further staging examinations did not show any other lesions suitable for a biopsy. A 18FDG positron emission tomography scan showed pathologic glucose metabolism, especially within the atrial septum and in two suspiciously sized lymph nodes (Fig 2). A mediastinoscopy was performed with negative results for all biopsies taken. As histology is considered to be crucial for all possible treatment strategies, it was decided that a histologic sample should be obtained by TEE-guided transvenous endocardiac biopsy (Fig 3). The few samples taken revealed the histology of a diffuse large cell B-cell lymphoma with a positive stain for CD20 and CD79 (Fig 4). Treatment with cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy was begun. An early control TEE scan 10 days after the first course of chemotherapy showed a massive reduction of the size of the mass. This result was confirmed by an early control of the 18FDG positron emission tomography scan 21 days after the application of the first course of chemotherapy. In this control scan the standardized uptake value decreased from 23.1 to 5.3 after the first course of chemotherapy. After three courses of chemotherapy, the patient achieved a complete remission.
Malignant lymphoma of the heart is a very rare disorder making up less than 1% of all lymphomas.1,2 Presenting symptoms are usually chest pain, congestive heart failure, pericardial effusions or, as in our patient, arrhythmias. The clinical course is characteristically acute in onset and short in duration, with a high rate of early death. Prolonged survival has been reported after chemotherapy, but prognosis remains poor. As has been shown in a recent review of the literature, 60% of patients die, whatever treatment is given.3,4 Histologic work-up is mandatory in patients presenting with myocardial masses because 75% of all myocardial tumors are benign. Primary malignant tumors are of sarcomatous or mesenchymal origin in 40% to 60% of the cases, while malignant lymphomas make up only 2% to 6% of tumors arising in the heart. In most of the patients, the diagnosis is obtained by biopsy of cardiac tissue during an explorative thoracotomy or mediastinoscopy. A cytologic sample of pericardial effusion may reveal the diagnosis, but it may be difficult to differentiate malignant lymphoma from benign reactive lymphocytosis. Transvenous endocardiac biopsy under simultaneous echocardiographic guidance is another possibility in obtaining cardiac tissue.5 Endomyocardial biopsy is routinely done using fluoroscopy only to diagnose myocarditis, amyloidosis, inflammatory diseases, or storage diseases. The risk is minimal. Perforation can be expected in about 0.5% of cases. In this case, however, the biopsy site is not the interventricular, but the atrial septum. For this unusual and potentially dangerous biopsy site with a much higher risk of perforation, TEE guidance is mandatory and allows correct positioning of the biopsy forceps.
Editor's Note
Authors' Disclosures of Potential Conflicts of Interest
REFERENCES 1. Sutcliffe SB, Gospodarowicz MK: Primary extranodal lymphomas, in Canellos GP, Lister TA, Sklar JL (eds): The Lymphomas. Philadelphia, W.B. Saunders Company, 1998, pp 449-479
2. 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 3. Ceresoli GL, Ferreri AJM, Bucci E, et al: Primary cardiac lymphoma in immunocompetent patients. Cancer 80:1497-1506, 1997[CrossRef][Medline] 4. Chalabreyesse 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] 5. Daus H, Bay W, Harig S, et al: Primary lymphoma of the heart: Report of a case with histological diagnosis of the transvenously biopsied intracardiac tumor. Ann Hematol 77:139-141, 1998[CrossRef][Medline]
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Copyright © 2004 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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