Jpn J Clin Oncol 2001;31(5)217–220
Complete Atrio-ventricular Block as a Major Clinical Presentation of the Primary Cardiac Lymphoma: a Case Report Cheng-Jeng Tai1, Wei-Shu Wang1, Ming-Teng Chung2, Jin-Hwang Liu1, Chih-Yao Chiang3, Chueh-Chuan Yen1, Frank Sheng Fan1, Tzeon-Jye Chiou1 and Po-Min Chen1 1Division
of Medical Oncology, Department of Medicine, Veterans General Hospital – Taipei and National Yang-Ming University School of Medicine, Taipei, 2Division of Pathology and 3Division of Cardiac Surgery, Department of Surgery, Cheng-Hsin Rehabilitation Medical Center, Taipei, Taiwan Received September 22, 2000; accepted January 30, 2001
It is uncommon for malignant lymphomas to present primarily with cardiac invasion as the main clinical feature. What we are interested in is not only where the disease is, but also those symptoms that it may induce. Sudden onset of complete atrio-ventricular block is one of the most common clinical presentations. Cardiac tamponade is another common disease entity which, if it locates over outlets of great vessels, may also exert symptoms of obstructive vessels. Diagnosis can be made by needle aspiration under the guidance of transcutaneous or transesophageal echocardiography. Both CT scan and MRI play positive roles in the diagnosis of cardiac lymphomas, and the latter can even provide much more image information than the former. We report a 70-year-old male with primary cardiac lymphoma with initial clinical pictures of sudden onset of complete atrio-ventricular block. Chemotherapy was utilized with cyclophosphamide, vincristine and prednisolone (COP) initially for four courses and followed by adding doxorubicin (CHOP) for another three courses. The patient was still in remission status after treatment for 2 years. Key words: cardiac lymphoma – chemotherapy – complete AV block
INTRODUCTION
CASE REPORT
It is uncommon for malignant lymphoma to invade the cardiac tissues, and therefore most cardiac lymphomas are found by postmortem examination without main clinical presentations. The heart as the primary occurring site where malignant lymphomas present, although extremely rare, still occasionally happens. Nevertheless, the most deleterious occurrence is that they result in a high mortality rate owing to the advanced involvement of the myocardial tissue (1). Clinical appearances of primary cardiac lymphomas vary from electrical conductance of the heart to obstructive signs in the great vessels. Experience with treating primary cardiac lymphomas is limited because there is little published material regarding the chemotherapeutic regimens particular for this disease (2).
A 70-year-old male was sent to Cheng-Hsin Rehabilitation Medical Center in September 1998 because of abdominal pain. On admission, blood pressure was 108/68 mmHg with pulse rate 60–121 BPM in normal sinus rhythm. His family reported no history of systemic diseases except elevated blood sugar and occasional epigastralgia in the past decades. On physical examination, the cardiovascular system and respiratory system were normal and no lymphadenopathy or hepatosplenomegaly presented on abdominal palpitation. Tenderness and eminent rebounding pain were diffuse on the abdomen, mainly over the middle to upper quadrant. Laboratory data revealed that the white blood cell count was 8200/mm3, hemoglobin 13.4 g/dl, platelets 254 000/mm3 and the differential count of white blood cells was neutrophils 61%, lymphocytes 27.9% and monocytes 11.1%. On serum biochemical analysis, the LDH was 235 U/l, total protein 5.9 g/dl, albumin 4.0 g/dl and the renal and liver function tests were normal. The initial chest X-ray showed abnormally enlarged right atrium and he then underwent a baseline ECG with normal sinus rhythm (Fig. 1). Abdominal sonography at that time revealed accumulated fluid in the abdominal cavity. He then underwent exploratory laparotomy with segmental resection of the ileum and was diagnosed as perforation with peritonitis of the ileum. The
For reprints and all correspondence: Po-Min Chen, Division of Medical Oncology, Department of Medicine, Veterans General Hospital – Taipei, 201 Sec. 2 Shih-Pai Road, Pei-Tou, 112, Taipei, Taiwan. E-mail:
[email protected]
© 2001 Foundation for Promotion of Cancer Research
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Figure 1. Baseline EKG taken on the patient’s arrival at Cheng-Hsin Rehabilitation Medical Center.
Figure 2. EKG showing complete AV block on occurrence.
pathology reported as autolytic changes and necrotic inflammation without evidence of malignancy. While in the recovery period after operation for peritonitis, this patient suffered a sudden onset of palpitation and chest discomfort. In the meantime, the ECG showed complete atrio-ventricular block (Fig. 2). A permanent pacemaker of DDD mode was implanted to correct for electrical generation failure. Echocardiography was therefore performed and he was by accident found to have a mass located over the right atrium (Fig. 3). Exploratory sternotomy with pericardial tumor biopsy was done and revealed multiple hard nodular tumors seeding on the anterior surface of the pericardium of the right atrium. As it was not possible to resect the whole tumor, a piece of tumor with a size of 1 ´ 0.5 ´ 0.4 cm3 was biopsied. Immunohistochemical stains of keratin, desmin, vimentin and actin of smooth muscle were all negative. CD3, MT1, L26 and LCA stains were done and found to be positive for L26 and LCA but negative for the rest, which indicated a malignant lymphoma of diffuse large B cell lineage (Fig. 4). He was then referred to Taipei Veterans
General Hospital for further treatment. We performed staging with chest and abdominal computed tomography, with negative findings except for the right atrium tumor. Bone marrow examination revealed no involvement of lymphoma cells. We adopted cyclophosphamide and vincristine (COP) as initial therapeutic modality for four courses with a favorable response and then added doxorubicin (CHOP) for another three courses. One year later, we performed another echocardiogram and found that the tumor had vanished (Fig. 5). Although the reason for inducing complete atrio-ventricular block had disappeared, we did not try to remove the pacemaker, which was still working well, because the patient was unwilling to undergo any invasive procedure. Two years after treatment, no relapse was found on follow-up.
DISCUSSION Primary cardiac lymphoma is a rare clinical entity and hardly ever diagnosed antemortem (3). Of 386 primary cardiac tumors
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Figure 3. Echocardiography reveals a mass lesion over the right atrium, as indicated by the arrow.
Figure 5. Echocardiography showing no residual mass lesion over the right atrium after completing chemotherapy.
and cysts of the heart reported by the Armed Forces Institute of Pathology, only seven (2%) were primary malignant lymphomas (4). Secondary lymphomatous involvement of the heart is much more common and has been reported as between 16 and 28% of extracardiac lymphomas (5). Primary cardiac lymphomas usually present a variety of clinical manifestations and are nearly always fatal (6). The two most commonly encountered clinical manifestations of primary cardiac lymphoma are pericardial effusion and heart failure (Table 1) (2,3,5). In this case, the main cardiac manifestations were complete atrioventricular block and congestive heart failure (7,8). A definite diagnosis is usually difficult to make. However, because the mass feature in the heart is often characteristic, echocardiography is therefore a good non-invasive diagnostic tool (9). Other than echocardiography, CT scanning and MR imaging also provide valuable information (6). So far, numerous modalities including gallium scan and blood pool isotopic
studies have been applied to aid in diagnosing cardiac lymphomas (3,6). Thallium scan is of limited value because no thallium uptake defect can be found (10). Ultrasound-guided percutaneous needle biopsy has been an efficient method for obtaining cardiac tissues (11,12,13). In this case, his condition was found accidentally owing to complete AV block as the initial clinical presentation. Therefore, he underwent an echocardiogram and was revealed to have a right heart mass. Open-heart surgery was performed with the suspicion of cardiac myxoma. A frozen section during the operation helped us in diagnosing primary cardiac lymphoma. Several therapeutic modalities have been reported, including chemotherapy with COP (cyclophosphamide, vincristine, prednisolone), CHOP (cyclophosphamide, anthracycline, vin-
Figure 4. Photomicrograph showing a microscopic view of the cardiac tumor (H&E stain, ´400). This picture depicts the cytological characteristics of large lymphocytes with abundant cytoplasm, vesicular nuclei and visible nucleoli.
Table 1. Summary of primary cardiac lymphomas previously reported in the literature Reference
Case Clinical presentation No.
Curtsinger et al. (5)
1
Left hemiparesis, right heart failure
Hwang et al. (14)
1
SVC syndrome, pericardial effusion
Roberts et al. (8)
1
Congestive heart failure
Roller et al. (15)
1
SVC syndrome; arrhythmia
Zaharia and Gill (2)
1
Pericardial effusion
Chao et al. (16)
1
Pericardial effusion
Ito et al. (17)
2
1 with pericardial effusion and CRBBB, 1 with pericardial effusion and endocarditis
Chim et al. (18)
2
Pericardial effusion, right heart failure
Nakata et al. (3)
1
Pericardial effusion
Makishima et al. (19)
1
Heart failure
Tai et al. (this work)
1
Complete AV block, congestive heart failure
AV, atrio-ventricular; CRBBB, complete right bundle branch block; SVC, superior vena cava.
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cristine, prednisolone), M-BACOD (methotrexate, bleomycin, anthracycline, cyclophosphamide, vincristine, dexamethasone), ESHAP (etoposide, methylprednisolone, cytarabine, cisplatin), radiation, resection and heart transplantation (1,13). Chemotherapy remains the best choice for treating this disease. Considering the possible consequence of cardiac rupture if anthracycline-containing agents are included in first-line medication, we therefore adopted COP as an alternative option. In conclusion, even though primary cardiac lymphoma is a rare disease, it is still possible to cure it by applying appropriate and adequate chemotherapy after diagnosis.
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