European Journal of Echocardiography 2003 4(4):327-330; doi:10.1016/S1525-2167(03)00002-7
© 2003 by European Society of Cardiology
Copyright © 2003, The European Society of Cardiology
Primary Cardiac Lymphoma
J Timperley*,
A.R.J Mitchell and
H Becher
Department of Cardiology, The John Radcliffe Hospital, Headington, Oxford, UK
* Address correspondence to: Dr. Jonathan Timperley, Department of Cardiology, The John Radcliffe Hospital, Headington, Oxford OX3 9DU, UK. Tel: +44-1865-221734; Fax: +44-1865-220585. cardiology.registrars{at}orh.nhs.uk
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Abstract
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We present a case of primary cardiac lymphoma, which is a rare
condition compared with secondary metastatic involvement and
is associated with poor prognosis. This case demonstrates the
use of transthoracic echocardiography for the assessment of
tumour regression in response to chemotherapy.
Keywords: cardiac lymphoma; transthoracic echocardiography; chemotherapy
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Patient History
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A 65-year-old man was admitted with a 3-month history of increasing
exertional dyspnoea and 14 kg weight loss. He was a previous
smoker and had a long history of industrial asbestos exposure
and a 3-year history of hypertension treated with diuretics.
On examination, he was cachectic with an elevated jugular venous pressure, soft heart sounds and crepitations at right lung-base. He had no palpable lymphadenopathy, hepatomegaly or splenomegaly. His ECG showed sinus tachycardia with T wave inversion in leads V2–6. His chest radiograph showed increased shadowing at the right base only. Blood tests revealed an elevated LDH 845 IU/l (110–250 IU/l) and CRP 97 mg/l. Full blood count, other biochemical and tumour markers were unremarkable. Transthoracic echocardiography (Figs. 1–3
) showed a thickened free wall of the right ventricle with a mass within right atrium and ventricle extending into the right ventricular outflow tract. The mass also extended around the aortic root. There was a small global pericardial effusion. Right ventricular function was severely impaired.
A CT scan of the thorax was performed, which confirmed a mass
encasing the heart and invading the right ventricle. At the
aortic root the mass was seen to enclose the left main coronary
artery. The CT scan also demonstrated numerous pulmonary emboli
especially affecting the right lower lobe. There was no mediastinal
lymphadenopathy, hepatomegaly or splenomegaly. A CT guided biopsy
was performed and the histology revealed a B-cell non-Hodgkin's
lymphoma.
He was treated with chemotherapy comprising cyclophosphamide, doxorubicin, vincristine and prednisolone. Transthoracic echocardiography 1 week following his initial chemotherapy showed no change and so his chemotherapy regime was changed to cyclophosphamide, doxorubicin, vincristine and methotrexate. Weekly imaging over the following 3 weeks revealed resolution of the pericardial effusion, a reduction of the tumour mass in the right ventricle and atrium and an improvement in right ventricular function. Correspondingly, his clinical condition improved. A parasternal short-axis view 1 week into this new regime is shown in Fig. 4.
Unfortunately, 6 weeks following his initial admission he developed
respiratory failure and required ventilation. Transoesophageal
echocardiography showed that the tumour mass continued to regress
with no significant effusion, and it was felt that the cause
of the respiratory failure was pulmonary tumour embolisation.
After ventilation for 10 days he was transferred to the haematology
ward, but he gradually deteriorated and died 4 months following
admission.
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Discussion
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Although metastatic cardiac involvement of lymphomas is common
(25%)
[1], primary cardiac lymphoma (extranodal involving only
the heart and pericardium) is a rare condition associated with
poor prognosis, with most patients dying before chemotherapy
can be started. There is an increasing frequency of this condition
in patients with immunosuppressive conditions, including AIDS
[2].
Symptoms are related to the position of the tumour. A relatively
common finding is atrioventricular block ranging from first
to third degree block
[3]. Although chemotherapy can lead to
significant tumour regression, early post-chemotherapy death
may be related to massive pulmonary emboli, refractory heart
failure and cardiac arrhythmias
[4].
Echocardiography is the most sensitive imaging technique, although a tissue diagnosis is still required[5]. In this case due to the large mass extending around the aortic root, a CT guided biopsy was performed. Other techniques include transoesophageal guided transvenous biopsy[6] and percutaneous intracardiac biopsy with combined transoesophageal and fluoroscopic guidance[7]. The access used will depend on local expertise and positioning of the tumour. Cytology of pericardial fluid is often negative[8]. Recently, myocardial contrast echocardiography has been used to help differentiate tumour from thrombus[9].
This case and others[10] demonstrate the ability of transthoracic imaging to assess tumour response to chemotherapy, aiding the physician to make decisions regarding changes in chemotherapy regime depending on the response of the tumour.
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References
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- Robert W.C., Glancy D.L., De Vita W.T. Heart in malignant lymphoma: a study of 196 autopsy cases. Am J Cardiol (1968) 22:85–107.[CrossRef][Web of Science][Medline]
- Holladay A.O., Siegel R.J., Schwartz D.A. Cardiac malignant lymphoma in acquired immune deficiency syndrome. Cancer (1992) 70:2203–2207.[CrossRef][Web of Science][Medline]
- Tai C.J., Wang W.S., Chung M.T., Liu J.H., Chiang C.Y., Yen C.C., Fan F.S., Chiou T.J., Chen P.M. Complete atrio-ventricular block as a major clinical presentation of the primary cardiac lymphoma: a case report. Jpn J Clin Oncol (2001) 31:217–220.[Abstract/Free Full Text]
- Rolla G., Bertero M.T., Pastena G., Tartaglia N., Corradi F., Casabona R., Motta M., Caligaris-Cappio F. Primary lymphoma of the heart. A case report and review of the literature. Leuk Res (2002) 26:117–120.[CrossRef][Web of Science][Medline]
- Tighe D.A., Anene C.A., Rousou J.A., King A.K., Engelman R.M. Primary cardiac lymphoma. Echocardiography (2000) 17:345–347.[CrossRef][Web of Science][Medline]
- Unger P., Kentos A., Cogan E., Renard M., Crasset V., Stoupel E. Primary cardiac lymphoma: diagnosis by transvenous biopsy under transesophageal echocardiographic guidance. J Am Soc Echocardiogr (1998) 11:89–91.[CrossRef][Web of Science][Medline]
- Jurkovich D., de Marchena E., Bilsker M., Fierro-Renoy C., Temple D., Garcia H. Primary cardiac lymphoma diagnosed by percutaneous intracardiac biopsy with combined fluoroscopic and transesophageal echocardiographic imaging. Catheter Cardiovasc Interv (2000) 50:226–233.[CrossRef][Web of Science][Medline]
- King D.T., Nieberg R.K. The use of cytology to evaluate pericardial effusions. Ann Clin Lab Sci (1979) 9:18–23.[Abstract]
- Lepper W., Shivalkar B., Rinkevich D., Wei K. Assessment of the vascularity of a left ventricular mass using myocardial contrast echocardiography. J Am Soc Echocardiogr (2002) 15:1419–1422.[CrossRef][Web of Science][Medline]
- Laine O.K., Groundstroem K.W., Koivunen E.A. Echocardiographic imaging of right ventricular outflow obstruction during chemotherapy of mediastinal lymphoma. Echocardiography (1999) 16:1–2.[CrossRef][Web of Science][Medline]
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