Primary Cardiac Lymphoma: Diagnosis by Transvenous Biopsy Under Transesophageal Echocardiographic Guidance

Primary Cardiac Lymphoma: Diagnosis by Transvenous Biopsy Under Transesophageal Echocardiographic Guidance Philippe Unger, MD, Alain Kentos, MD, Elie Cogan, MD, Marc Renard, MD, Vincent Crasset, MD, and Eric Stoupel, MD, Brussels, Belgium

A 64-year-old woman presenting with dizziness and atrioventricular conduction disturbances was found to have a right atrial mass by two-dimensional transthoracic echocardiography. Transesophageal echocardiography allowed further delineation of the tumor and safe perfor-

Despite a recent frequency increase in immunocompromised patients, primary cardiac lymphoma remain extremely rare and antemortem diagnosis has seldom been reported.1 We report a patient in whom primary cardiac non-Hodgkin’s B cell lymphoma was diagnosed with transvenous biopsy performed under transesophageal echocardiographic guidance, thereby obviating the need for surgery. A 64-year-old woman was admitted to our institution because of dizziness. Sixteen years earlier, quadrantectomy followed by radiotherapy had been performed for breast carcinoma. Five months earlier, hemorrhagic pericardial effusion had been found. A comprehensive evaluation including bacteriologic and cytologic examination of pericardial fluid and open pericardial biopsy failed to allow a specific diagnosis. Ten days before admission, first-degree atrioventricular block had been found on routine electrocardiogram (ECG). The patient gradually began to complain of dizziness in the upright position. On admission, the pulse was slow and irregular; physical examination was otherwise unremarkable. Atrial fibrillation with a ventricular response of 36 beats/min was observed on ECG. A temporary pacemaker was inserted. Transthoracic echocardiography disclosed a nonmobile posteroinferior right atrial mass and inferior vena cava plethora but failed to accurately delineate the tumor. On transesophageal echocardiography, a marked interatrial thickening was observed, exhibiting a lipomatous hypertrophy-like appearance (FigFrom the Departments of Cardiology and Internal Medicine, Erasme Hospital, Universite´ Libre de Bruxelles. Reprint requests: P. Unger, MD, Cardiology Department, Erasme Hospital, 808, Route de Lennik, B-1070 Brussels, Belgium. Copyright © 1998 by the American Society of Echocardiography. 0894-7317/98 $5.00 1 0 27/4/83884

mance of transvenous biopsy, thereby obviating the need for surgery. Pathological examination of the biopsy specimen as well as the absence of extracardiac location established the diagnosis of primary cardiac lymphoma. (J Am Soc Echocardiogr 1998;11:89-91.)

ure 1, A). A large lobulated tumor was visualized in the posteroinferior right atrium, extending to the inferior left atrial wall, compressing the coronary sinus and the inferior vena cava admission (Figure 1, B). Magnetic resonance imaging, gallium scintigraphy, bone biopsy, and thoracic and abdominopelvic computed tomography scans failed to show any extracardiac location. Transvenous biopsy was undertaken through a right internal jugular vein approach, under transesophageal guidance, using a 5 MHz multiplane transducer. Echocardiography allowed the bioptome to be accurately positioned (Figure 2); particular attention was paid to visualize the forceps in various planes to confirm its correct positioning over the mass. Three samples were obtained in three different tumor areas. Microscopic examination showed a diffuse large cell non-Hodgkin’s B cell lymphoma. A human immunodeficiency virus antibody test was negative. Six cycles of combination chemotherapy (cyclophosphamide, novantrone, vincristine, and prednisone) allowed the complete disappearance of the tumor, confirmed by a repeat transesophageal echocardiography performed 6 weeks later and restoration of sinus rhythm with normal PR interval. A 6-month follow-up period was uneventful. Because the tumor was extranodal, involving only the heart and pericardium, our case met the current criteria for a primary cardiac lymphoma.1 Cytologic analysis performed on pericardial effusion was negative, but this is not unusual with lymphoma.2 Although secondary cardiac involvement occurs in up to 24% of post mortem evaluation of lymphoma cases, primary cardiac lymphoma is extremely rare, with fewer than 40 cases in immunocompetent patients described up to 1996 in the English literature.1 The clinical presentation is related to the location of the tumor.1 In our patient, the extension toward the coronary sinus and the triangle of 89

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Figure 1 A, Transesophageal echocardiogram in horizontal plane four-chamber view showing tumoral thickening of the interatrial septum (arrow). B, In the lower right atrium (RA), a large polylobulated tumor is seen, compressing the inferior vena cava (IVC) and the coronary sinus (CS). LA, Left atrium.

Koch likely explains the occurrence of reversible atrioventricular conduction disturbances. Histologic diagnosis of intracardiac masses may be obtained by surgical exploration, cytologic examination of pericardial fluid, or transvenous biopsy, usually under fluoroscopic guidance.3,4 However, the lack of direct visualization may impede the obtainment of tissue. Transthoracic echocardiography may overcome this limitation but occasionally fails to provide adequate images, particularly in the posterior cardiac structures. Two cases of right atrial tumors

diagnosed with transesophageal echocardiography– guided transvenous biopsy have been reported, including one nonprimary cardiac lymphoma.5,6 Considering the history of our patient, the concern that the tumor may be a breast carcinoma metastasis or a cardiac sarcoma prompted us to favor a nonsurgical approach. As the right atrial free wall appeared unaffected and thin, fluoroscopic guidance alone may have been not only unsuccessful but also hazardous, with a risk of wall perforation and cardiac tamponade. Transesophageal guidance provides high-resolution images to guide

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Figure 2 Transesophageal echocardiogram in vertical plane showing the bioptome (arrows) in the right atrium during biopsy. SVC, Superior vena cava; T, tumor.

bioptome placement. Furthermore, compared with fluoroscopy-guided biopsy, it reduces radiation exposure and may allow early detection of biopsy-related complications such as pericardial effusion. Despite a theoretic risk of dislodging tumor material, transvenous biopsy should be considered when chemotherapy is expected to be the optimal therapeutic approach, and transesophageal echocardiography has the potential to increase the safety of the procedure. REFERENCES 1. McCallister HA, Fenoglio JJ. Tumors of the heart and great vessels. Atlas of tumor pathology. 3rd Series, fascicle 16. Washing-

ton, DC: Armed Forces Institute of Pathology; 1996. p. 171-9. 2. King DT, Nieberg RK. The use of cytology to evaluate pericardial effusions. Ann Clin Lab Sci 1979;9;18-23. 3. Flipse TR, Tazelaar HD, Holmes DR Jr. Diagnosis of malignant cardiac disease by endomyocardial biopsy. Mayo Clin Proc 1990;65:1415-22. 4. Basso C, Stefani A, Calabrese F, Fasoli G, Valente M. Primary right atrial fibrosarcoma diagnosed by endocardial biopsy. Am Heart J 1996;131:399-402. 5. Azuma T, Ohira A, Akagi H, Yamamoto T, Tanaka T. Transvenous biopsy of a right atrial tumor under transesophageal echocardiographic guidance. Am Heart J 1996;131: 402-4. 6. Salka S, Siegel R, Sagar KB. Transvenous biopsy of intracardiac tumor under transesophageal echocardiographic guidance. Am Heart J 1993;125:1782-04.

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