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Originally published as JCO Early Release 10.1200/JCO.2008.19.4803 on November 24 2008 © 2008 American Society of Clinical Oncology.
Primary Cardiac Lymphoma Presenting As Sick Sinus Syndrome
Cardiology Operative Unit, S. Andrea Hospital, La Spezia, Italy
Department of Neurology, S. Andrea Hospital, La Spezia, Italy
Department of Oncohaematology, Felettino Hospital, La Spezia, Italy
Laboratory of Pathologic Anatomy, S. Andrea Hospital, La Spezia, Italy A 76-year-old woman with history of arterial hypertension and recent-onset dyspnea was visited at our laboratory of echocardiography. Twenty weeks earlier, she had undergone dual-chamber rate-adaptive pacemaker implantation at our department because of syncope with evidence of sick sinus syndrome, a cardiac rhythm disturbance secondary to sinus node dysfunction that is associated with several types of bradycardia. An echocardiogram performed 2 days before implantation had shown no significant abnormalities. At the time of implantation, the patient was asymptomatic and in good general condition, catheter positioning procedure had been completed with no technical difficulties, and chest x-ray had confirmed correct positioning of the electrodes. The patient had been discharged, but worsening dyspnea had occurred during the following weeks. At the present examination, the woman was dyspneic and in poor general condition. Jugular turgor and peripheral edema were present. The comparison of serial chest x-rays showed progressive deformation of the cardiac silhouette in comparison with that observed after pacemaker implantation. Transthoracic echocardiography evidenced a large mass obliterating the right atrium and causing functional tricuspid stenosis. Transesophageal echocardiography allowed better definition of the mass, which was characterized by a grossly ellipsoidal shape (8.3 x 6.5 cm), smooth surface, irregular architecture, and inhomogeneous echogenicity (Fig 1, panels A-B, and movies 1 and 2 [supplemental video, online only]; AO, aortic valve; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; VE, ventricular electrode). The mass entirely surrounded both electrodes, and extended cranially into an occluded superior vena cava (SVC). Color Doppler imaging evidenced no detectable flow from the SVC, and revealed a vascularized architecture (Fig 1, panels C-D, and movies 3 and 4 [supplemental video, online only]; AE, atrial electrode; IVC, inferior vena cava; other abbreviations as previously described). Contrast-enhanced chest computed tomography (CT) confirmed right atrial obliteration and SVC occlusion by a large vascularized mass (8.5 x 6.5 x 8.0 cm; estimated volume 237 cm3) encompassing both electrodes and infiltrating adjacent tissues (Fig 2; dotted white arrows: infiltrative margins; black arrows: vascular images within the mass; AA, ascending aorta; DA, descending aorta; other abbreviations as in Fig 1). A cardiac malignant tumor was suggested. Extensive diagnostic work-up yielded no evidence of extracardiac neoplastic manifestations. Because of the highly compromised conditions of the patient, surgery was considered not feasible. Chemotherapy and supportive treatment were initiated, but patient's conditions rapidly deteriorated with refractory right-sided heart failure. A second CT, performed 9 days after the first one, showed further increase in mass dimensions (9.2 x 7.0 x 8.5 cm; volume 287 cm3; relative increase 24%; mean growth rate 6.1 cm3/d). On day 25 after admission in our laboratory, the patient died. Autopsy (Fig 3, panels A-B) confirmed the presence of a large and vascularized right atrial mass (asterisks) characterized by friable and hemorrhagic consistency, thrombotic extension into the SVC (white arrows) and encasement of both electrodes. Histology showed extensive lymphocyte infiltration (Fig 3, panel C), and immunohistochemical analysis revealed staining for CD 20 (Fig 3, panel D), showing the mass to be a large B-cell primary cardiac lymphoma (PCL).
PCL represents 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas.1 It occurs more commonly in immunocompromised patients, particularly in HIV-positive individuals, whereas its detection in immunocompetent subjects is exceedingly rare.2,3 Clinical presentations may depend on flow obstruction, infiltration of adjacent tissues, or tumor embolization, and include heart failure, arrhythmias, atrioventricular disturbances, pericardial effusion, SVC syndrome, and stroke.4,5 Sick sinus syndrome has been reported as a complication of lymphomas with secondary myocardial involvement,6 but its occurrence as first presentation of PCL is exceptional. The prognosis depends on tumor stage and location, comorbidities, and the immunological status of the patient, but early diagnosis is fundamental to allow prompt initiation of aggressive treatment.7 Although in most cases this may be achieved by transthoracic echocardiography, the nonspecific clinical presentations often lead to diagnostic delay. In this view, tumor growth rate represents a key aspect in affecting the feasibility of early diagnosis. These images illustrate the massive growth of a PCL characterized by unusual presentation and fatal evolution over less than 24 weeks. Because neither transesophageal echocardiography nor chest CT was performed at the time of pacemaker implantation, the effective stage of tumor mass at that time cannot be established. Nonetheless, the following elements support the hypothesis of an extremely high growth rate: (1) the absence of right atrial masses by transthoracic echocardiography immediately before implantation; (2) the evidence of no significant technical difficulties during the implantation procedure; (3) the successive onset of symptoms due to right-sided heart failure; (4) the progressive changes in the cardiac silhouette at serial chest x-rays performed after the implantation; (5) the echocardiographic, radiological, and authoptic finding of pacemaker electrodes included within the mass; and (6) the considerable 9-day increase in mass volume estimated by serial CT. Despite improvements in outcome due to the wide availability of echocardiography,7 the high growth capability may still represent a major clinical problem for the early diagnosis of PCL. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
NOTES published online ahead of print at www.jco.org on November 24, 2008. REFERENCES
1. Rockwell L, Hetzel P, Freeman JK, et al: Cardiac involvement in malignancies: Case 3—Primary cardiac lymphoma. J Clin Oncol 22:2744-2745, 2004 2. Nguyen DT, Meier CR, Schneider D: Primary cardiac lymphoma mimicking left atrial myxoma in an immunocompetent patient. J Clin Oncol 26:150-152, 2008 3. 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] 4. Binder J, Pfleger S, Schwarz S: Images in cardiovascular medicine: Right atrial primary cardiac lymphoma presenting with stroke. Circulation 110:e451-e452, 2004 5. Beckwith C, Butera J, Sadaniantz A, et al: Diagnosis in oncology: Case 1—Primary transmural cardiac lymphoma. J Clin Oncol 18:1996-1997, 2000 6. Kamimura M, Tanabe N, Hojo M, et al: Malignant lymphoma demonstrating sick sinus syndrome. Intern Med 37:463-466, 1998[CrossRef][Medline] 7. Faganello G, Belham M, Thaman R, Blundell J, Eller T, Wilde P: A case of primary cardiac lymphoma: Analysis of the role of echocardiography in early diagnosis. Echocardiography 24:889-892, 2007[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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