International Journal of Cardiology 113 (2006) e26 – e29
www.elsevier.com/locate/ijcard

Letter to the Editor

Successful treatment of primary cardiac B-cell lymphoma: Depiction at
multislice computed tomography and magnetic resonance imaging
Yuichi Sato a,⁎, Naoya Matsumoto a , Noriko Kinukawa b , Shinro Matsuo c , Sei Komatsu d ,Taeko Kunimasa a , Shunichi Yoda a , Shigemasa Tani a , Tadateru Takayama a , Yuji Kasamaki a ,Satoshi Kunimoto a , Satoru Furuhashi e , Motoichiro Takahashi e , Satoshi Saito aab

Department of Cardiology, Nihon University School of Medicine, 1-8-13 Kanda-Surugadai, Chiyoda-ku, Tokyo 101-8309, Japan
Department of Pathology, Nihon University School of Medicine, 1-8-13 Kanda-Surugadai, Chiyoda-ku, Tokyo 101-8309, Japanc
Department of Cardiovascular-Respiratory Medicine, Shiga University of Medical Science, Japand
Cardiovascular Division, Osaka Police Hospital, Osaka, Japane
Department of Radiology, Nihon University School of Medicine, Japan
Received 12 April 2006; accepted 15 July 2006

Abstract
Primary cardiac malignant lymphomas are extremely rare and the majority of lymphomas are aggressive B-cell lymphomas. We describe apatient with primary cardiac B-cell lymphoma presenting with superior vena caval syndrome and dyspnea. The tumors manifested as
hypoechoic immobile masses on echocardiography, poorly enhancing masses on contrast-enhanced multislice computed tomography and isointense masses on T1-weighted and hyper-intense masses on T2-weighted magnetic resonance images. Pathologic examination revealed thatthe mass was consistent with B-cell malignant lymphoma. Systemic chemotherapy together with monoclonal CD 20 antibody treatment was
initiated. There was marked regression of the tumor 4 days after the treatment and complete disappearance of the tumor after 8 days after thetreatment without episodes of systemic or pulmonary embolism.
© 2006 Elsevier Ireland Ltd. All rights reserved.
Keywords: Primary cardiac lymphoma; Multislice computed tomography; Magnetic resonance imaging; Monoclonal CD 20 antibody

1. Case report
A 77-year-old woman was referred to our hospital forbiopsy of her cervical lymphnode. She had had mild degree
fever, dyspnea on effort, facial and right arm edema andbilateral cervical lymphadenopathy, which started 2 months
earlier. On physical examination, there was a marked
dilatation of both jugular veins, and facial and right arm
edema. Laboratory examinations revealed that an increase in
C-reactive protein (2.9 mg/dl) and lactate dehydrogenase(360 IU/L). The hematologic findings were unremarkable.
Human immunodeficiency virus (HIV) was negative. Bonemarrow aspiration and biopsy revealed no infiltration of thelymphoma cells. Transthoracic echocardiography showed an⁎ Corresponding author. Tel.: +81 3 3293 1711; fax: +81 3 3295 1859.
E-mail address: yuichis@med.nihon-u.ac.jp (Y. Sato).
0167-5273/$ - see front matter © 2006 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijcard.2006.07.090

immobile, hypoechoic round mass in the right atrium and theleft atrium (Fig. 1a). Color Doppler imaging disclosed thatthe mass obstructed the drainage flow from the superior vena
cava into the right atrium (Fig. 1b). Contrast-enhanced
multislice computed tomography (MSCT) was performed
using a SOMATOM Volume Zoom (Siemens, Erlangen,Germany) with a gantry rotation of 500 ms/rotation andcollimation of 3.0 mm. MSCT showed masses which were
homogeneous and poorly enhanced (Fig. 2a–d). The rightcoronary involvement by the right atrial mass was also
depicted (Figs. 2a and 1b). The coronal image showed theobstruction of the superior vena cava–right atrium inflow
tract (Fig. 2d). Magnetic resonance imaging (MRI) was
performed by using an Intera Achieva (1.5 Tesla, Philips
Medical Systems, Netherlands). All the images were acquired by using navigator-guided, free-breathing sequences.
T1-weighted images (Fig 2e–g) showed homogeneousY. Sato et al. / International Journal of Cardiology 113 (2006) e26–e29

e27

Fig. 1. a: Echocardiography showing relatively hypoechoic masses in the right atrium (large arrow) and in the left atrium (small arrow), b: Color Doppler imaging
showing that the mass obstructs the superior vena cava–right atrium inflow tract.

masses (3 × 3 cm, x and y axis, respectively) in the rightatrium and in the left atrium (7 × 6 × 3 cm, x, y and z axis,
respectively) with signals equivalent to the myocardium. T2weighted images (Fig. 2h–j) revealed that the masses weremildly high-intense. A 67Ga scintigraphy showed abnormal
uptake in the heart (Fig. 2k,l). Microscopic examination of
the biopsy specimen from the right cervical lymphnodeshowed diffuse proliferation of large atypical lymphoid cellswith ovoid hyperchromatic nuclei and prominent nucleoli(Fig. 3a). Immunohistochemical investigations disclosed

negative reactivity with anti-keratin, anti-T cell, and positive
reactivity with CD 20 (Fig. 3b). Thus, the diagnosis of primary cardiac B-cell malignant lymphoma was confirmed andthe patient underwent systemic chemotherapy consisting of
cyclophosphamide, doxorubicin hydrochloride, vincristine
sulfate and prednisolone and monoclonal CD 20 antibody(Rituximab) (R-CHOP). Surprisingly, there was marked regression of the tumor 4 days after the initiation of R-CHOP
therapy, and almost complete disappearance of the tumor wasachieved 8 days after the R-CHOP therapy (Fig. 4). There

Fig. 2. Contrast-enhanced multislice computed tomographic images on the horizontal axis (a, b) demonstrating non-contrast-enhanced masses in the right atrium(large arrows) which surrounds the right coronary artery (RCA) and in the bottom of the left atrium (small arrow). The long axis image (c) showing the mass in
the left atrium (arrow). Coronal image (d) showing obstruction of the superior vena cava (SVC)–right atrium inflow tract by the tumor. T1-weighted magnetic
resonance images on the axial (e, f) and long-axis (g) planes showing iso-intensity masses in the right (large arrows) and left atrium (small arrows). The masses
are mildly high-intense on T2-weighted images (h–j). Planar (k) and tomographic (l) images of 67Ga scintigram showing high isotope uptake in the heart(arrows).

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Y. Sato et al. / International Journal of Cardiology 113 (2006) e26–e29

Fig. 3. (a) Histologic section from the cervical lymphnode showing large
abnormal cells with irregular nuclei (hematoxylin and eosin stain, ×600)
which positively stained for CD 20 antigen (b).

were no major complications such as systemic or pulmonary
embolism.
2. Discussion
Primary cardiac lymphoma, defined as a non-Hodgkin's
lymphoma involving only the heart and/or pericardium or as alymphoma with the bulk of the tumor located on the heart isextremely rare in immunocompetent patients, accounting for1.3% of all cardiac tumors [1]. In the reviewed cases, approximately 80% is a B-cell lymphoma of the diffuse cell type[2,3]. Primary cardiac lymphoma remains asymptomatic until
it produces a mass effect when the tumor obstructs cardiac

chambers and great vessels, pulmonary or systemic embolization, complete atrioventricular block and cardiac tamponade. Transthoracic echocardiography is the first choice ofdiagnostic modality for the diagnosis of primary cardiac
lymphomas, but because of its limited acoustic window, theextent of the tumor can not be fully evaluated. Contrastenhanced computed tomography (CT) and MRI haveadvantages over echocardiography because they provide
better contrast resolution and allow simultaneous visualizationof the great vessels, heart, pericardium, mediastinum and thelung. Furthermore, CT and MRI may represent information
useful for the differential diagnosis of tumors depending on
their CT density and/or MR signal intensity characteristics,
especially in cardiac lipoma, osteochondrosarcoma andpericardial cysts. Although reports describing CT characteristics of primary cardiac lymphoma are rare, primary cardiac
lymphoma is characterized by homogeneous and poorly
enhanced mass [4], as was found in our patient. MRI may beeven more advantageous to CT because it allows tissue
characterization of the tumor. MRI findings on primary cardiac lymphoma are scarce, but homogeneous, iso- or hypointensity on T1-weighted image and relatively hyper-intensity
on T2-weighted image have been observed [4,5]. In addition,
heterogeneity of the signal intensity on T2-weighted image
has been reported to be related with a malignancy grade oflymphoma [6]. Primary cardiac lymphoma, unlike othercardiac malignancies, responds to chemotherapy. In fact, there
was almost complete disappearance of the tumor 8 days after
the initiation of R-CHOP therapy in our patient without
systemic or pulmonary embolism. Thus, accurate diagnosis
by CT and MRI, and subsequent early treatment are of utmost
importance in the management of patients with suspectedprimary cardiac lymphoma.

Fig. 4. T2-weighted magnetic resonance images 4 days (a–c) and 8 days (d–f) after the initiation of R-CHOP therapy. Marked regression of the tumor wasobserved 4 days after the treatment (arrow) and complete disappearance of the tumor was observed 8 days after the treatment.

Y. Sato et al. / International Journal of Cardiology 113 (2006) e26–e29

References[1] Roberts WC. Primary and secondary neoplasm of the heart. Am J
Cardiol 1997;80:671–82.
[2] Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E. Primary
cardiac lymphoma in immunocompetent patients. Diagnostic andtherapeutic management. Cancer 1997;80:1497–506.
[3] Chim CS, Chan ACL, Kwong YL, Liang R. Primary cardiac lymphoma.
Am J Hematol 1997;54:79–83.

e29[4] Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MR imaging ofprimary cardiac malignancies. Radiograhics 1999;19:1421–34.
[5] Dorsay TA, Ho BV, Rovira JM, Armstrong AM, Brissette DM. Primary
cardiac lymphoma: CT and MR findings. J Comput Assist Tomogr
1993;17:978–81.
[6] Rehn S, Sperber GO, Nyman R, Glimelius B, Habgerg H, Hemmingsson A. Quantification of inhomogeneities in malignancy grading of nonHodgkin lymphoma with MR imaging. Acta Radiol 1993;34:3–9.

International Journal of Cardiology 109 (2006) 125 – 126
www.elsevier.com/locate/ijcard

Letter to the Editor

MDCT of the anomalous origin of the right coronary artery from theleft sinus of Valsalva as a single coronary artery
Yuichi Satoa,*, Makoto Ichikawaa, Mitsuyo Masubuchia, Shunichi Yodaa, Satoru Furuhashib,
Motoichiro Takahashib, Yasushi Koyamac, Satoshi Saitoa
aDepartment of Cardiology, Nihon University School of Medicine, 1-8-13 Kanda-Surugadai, Chiyoda-ku, Tokyo 101-8309, Japan
Department of Radiology, Nihon University School of Medicine, 1-8-13 Kanda-Surugadai, Chiyoda-ku, Tokyo 101-8309, Japanc
Division of Advanced Cardiac Imaging, Department of Cardiology, Cleveland Clinic Foundation, OH, USAb

Received 22 March 2005; accepted 26 March 2005
Available online 16 May 2005

Keywords: Coronary artery anomaly; MDCT; Virtual angioscopy

1. Case report
A 31-year-old man underwent multi-detector-row computed tomography (MDCT) coronary angiography becauseof episodes of chest pain followed by syncope in the earlymorning. He was referred from another hospital where his
Holter ECG had shown transient ST-segment elevation on
inferior extremity leads and sinus bradycardia. Conventional coronary angiography was performed but the rightcoronary artery (RCA) could not be visualized. Myocardial
perfusion scintigraphy showed no perfusion defect after
exercise. MDCT was performed using an Aquillion 16 (16detector-row, Toshiba Medical, Tokyo, Japan). The scan
protocol and image reconstruction method have beenreported previously [1]. The reconstructed data weretransferred to a computer workstation (M 900 quadra,
AMIN, Tokyo, Japan) for processing the surface volume
rendering and virtual angioscopic images. The volume
rendering image (Fig. 1a) showed that the RCA arose fromthe left sinus of Valsalva and coursed anteriorly between
the aortic root and the pulmonary artery. The axial image(Fig. 1b) showed an acute angled take-off of the RCA fromthe left main coronary artery. A 3D virtual angioscopic
image (Fig. 2) also demonstrated that the RCA originatedfrom the proximal portion of the left main coronary artery.

* Corresponding author. Tel.: +81 3293 1711; fax: +81 3295 1859.
E-mail address: yuichis@med.nihon-u.ac.jp (Y. Sato).
0167-5273/$ - see front matter D 2005 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijcard.2005.03.077

With hesitation about surgical treatment, the patient wasmaintained on oral medication by isosorbide dinitrateuneventfully.

2. Discussion
The anomalous origin of the RCA is a rare condition,
but has clinical importance because nonfatal or fatal

Fig. 1. (a) Volume rendering image showing the anomalous origin of theright coronary artery (arrow) which arises from the left sinus of Valsalva
and courses between the ascending aorta and the pulmonary artery.
Intramural course of the proximal portion of the right coronary arterywithin the aortic wall can not be observed on this image. (b) Axial image
shows the right coronary artery (arrow) arising from the proximal portion of
the left main coronary artery (LMCA).

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Y. Sato et al. / International Journal of Cardiology 109 (2006) 125 – 126

left main coronary artery and intramural course within theaortic wall. Although these findings suggest possible
causes of myocardial ischemia, our data could not clarify
the exact mechanism of myocardial ischemia because
MDCT images were obtained only in end-diastole, but notin systole during which ostial stenosis or compression of
the RCA within the aortic wall might have occurred. In
addition, the flap-like texture of the RCA orifice might
have been overlooked because spatial resolution of
MDCT was limited. The future development in the
MDCT hardware which will provide higher spatial
resolution and allow motion analysis during the wholecardiac cycle would be more informative for the evaluation of the mechanisms by which myocardial ischemia isprovoked in patients with the anomalous origin of the
RCA.

Fig. 2. Three-dimensional virtual angioscopic image showing a small
orifice of the right coronary artery (arrow) arising from the left main
coronary artery.

myocardial infarction and sudden death occur in up to
30% of patients [2 –4]. In the majority of patients, theRCA arises from the left sinus of Valsalva, separatelyfrom the left main coronary artery. On the other hand, theanomalous origin of the RCA from the left main coronary
artery as a single coronary artery is extremely rare [5].
The causes of myocardial ischemia in this anomaly
remain unclear, but the acute angle take-off and kinkingof the RCA as it arises from the left main coronary artery,flap-like closure of the abnormal coronary orifice,compression of the RCA when it courses within theaortic wall or between the aorta and the pulmonary artery,and spasm of the anomalous RCA have been thought tobe the possible mechanisms [6]. In our patient, MDCT
demonstrated acute angle take-off of the RCA from theReferences[1] Sato Y, Matsumoto N, Kato M, et al. Noninvasive assessment ofcoronary artery disease by multislice spiral computed tomography using
a new retrospectively ECG-gated image reconstruction technique:comparison with angiographic results. Circ J 2003;67:401 – 5.
[2] Engel HJ, Torres C, Page HL. Major variations in anatomical origin of
the coronary arteries: angiographic observations in 4250 patients
without associated congenital heart disease. Catheter Cardiovasc Diagn
1975;1:157 – 69.
[3] Yamanaka O, Hobbs RE. Coronary artery anomalies in 126 595 patients
undergoing coronary angiography. Catheter Cardiovasc Diagn
1990;21:28 – 40.
[4] Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital
coronary artery anomalies with origin from the wrong aortic sinus
leading to sudden death in young competitive athletes. J Am Coll
Cardiol 2000;35:1493 – 501.
[5] Gowda RM, Khan IA, Undavia M, Vasavada BC, Sacchi TJ. Origin of
all major coronary arteries from left sinus of Valsalva as a common
coronary trunk: single coronary artery. Angiology 2004;55:103 – 5.
[6] Roberts WC, Siegel RJ, Zipes DP. Origin of the right coronary arteryfrom the left sinus of Valsalva and its functional consequences: analysisof 10 necropsy patients. Am J Cardiol 1982;49:863 – 8.