Asian Cardiovasc Thorac Ann 2008;16:490-491
© 2008 Asia Publishing EXchange Ltd
Primary Right Atrial Angiosarcoma
Kirkpatrick Santo, FRCS,
Uday Dandekar, FRCS (CTh)
Department of Cardiothoracic Surgery, Queen Elizabeth Hospital, Birmingham, United Kingdom
For reprint information contact: Uday Dandekar, FRCS (CTh) Tel: 44 778 682 4219 Fax: 44 121 627 2541 Email: udandekar{at}yahoo.co.uk, Department of Cardiothoracic Surgery, Queen Elizabeth Hospital, Birmingham B15 2TH, UK.
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ABSTRACT
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A rare case of right atrial angiosarcoma is described in a 55-year-old man who was admitted with acute chest pain. Electrocardiography, cardiac enzymes, and chest radiography were negative. His pain settled and he was discharged, but readmitted 15 days later with clinical features of cardiac tamponade. Computed tomography demonstrated a large pericardial effusion. Emergency surgery was performed to excise a right atrial tumor, which histology confirmed to be an angiosarcoma.
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INTRODUCTION
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Primary cardiac tumors are very rare. Angiosarcomas are tumors of mesenchymal origin and account for 25% of malignant cardiac tumors. Because of early local and systemic spread, rarely do they present for surgical intervention.1
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CASE REPORT
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A 55-year-old man was admitted with acute onset of chest pain. In addition to routine investigations, he had an electrocardiogram, cardiac enzyme assays, and chest radiography. Investigations were inconclusive, and as the pain had settled, he was discharged home after 48 hours. He was readmitted 15 days later with severe shortness of breath and clinical features of cardiac tamponade. Transthoracic echocardiography and computed tomography of the chest confirmed a large pericardial effusion with a suspicious right atrial mass (Figure 1
). He was taken for an emergency exploratory median sternotomy. At surgery, approximately 800 mL of blood was drained from the pericardium. There was a bleeding tumor approximately 4 x 3 cm in size on the anterior surface of the right atrium, in close proximity to the atrioventricular groove, with a significant intraatrial component (Figure 1). Cardiopulmonary bypass was established with ascending aortic and bicaval venous cannulation. Systemic cooling to 28°C and topical cooling with intermittent antegrade cold blood cardioplegia were used for myocardial protection. The right atrium was opened close to the atrioventricular groove, taking care to protect the right coronary artery and the atrioventricular grove. There was tumor extension into the right atrium onto its lateral wall. All macroscopic tumor mass was removed with the adjacent right atrial wall. The subsequent defect (4 x 4 cm) in the atrial wall was repaired with a patch of bovine pericardium using 4/0 Prolene continuous suture. Separation from bypass and the postoperative course were uneventful. The patient was discharged on the 6th postoperative day. Histology confirmed angiosarcoma. He remained well and asymptomatic at the 6-month follow-up. However, long-term follow-up is essential, as prognosis and mid-term survival with this condition is guarded.
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DISCUSSION
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Because of early local and systemic dissemination, very rarely do patients with angiosarcomas undergo surgery. Our patient presented because of spontaneous rupture of the tumor and cardiac tamponade, and underwent successful surgical intervention.
Metastatic tumors are more common than the primary type, and benign tumors are more frequently found than malignant primary growths. Angiosarcoma of the heart involves the right atrium almost exclusively, but it has been reported in other cardiac chambers.2 Angiosarcomas of the heart grow rapidly, usually within the myocardial wall, which makes diagnosis difficult by various imaging methods. They are characterized by a tendency to bleed, often associated with pericardial effusion and cardiac tamponade.1,3 Many cases have been confirmed only at surgery for repeated pericardial effusion or at autopsy. Echocardiography is the most frequently used method, the transesophageal technique being preferred. The diagnosis may be suggested by computed tomography or magnetic resonance imaging. Myocardial biopsy is not advisable due to the friability of angiosarcoma.1 Treatment of angiosarcoma is controversial due to the poor prognosis. Surgical resection is indicated when no evidence of metastasis exists and when myocardial resection is reparative.4 Chemotherapy and radiation therapy may be indicated as adjuvant or preferential therapies, but their use is usually limited due to the poor physical condition of the patient. Survival ranges from 6 to 9 months, regardless of the treatment chosen.2 Cardiac transplantation has been performed, but use of immunosuppressant drugs is a limiting factor because it predisposes to tumor recurrence and metastases.5 Cardiomyoplasty is a surgical alternative when partial ventricular resection is required.6 This case highlights the need for awareness among clinicians of the possibility of this rare condition.
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REFERENCES
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- Corso RB, Kraychete N, Nardeli S, Moitinho R, Ourives C, Silva RM, et al. Spontaneous rupture of a right atrial angiosarcoma and cardiac tamponade. Arq Bras Cardiol 2003;81:611–3.[Medline]
- Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992;103:655–65.[Abstract]
- Ohri SK, Nihoyannopoulos P, Taylor KM, Keogh BE. Angiosarcoma of the heart causing cardiac rupture: a cause of hemopericardium. Ann Thorac Surg 1993;55:525–8.[Abstract]
- McFadden PM, Ochsner JL. Atrial replacement and tricuspid valve reconstruction after angiosarcoma resection. Ann Thorac Surg 1997;64:1164–6.[Abstract/Free Full Text]
- Baay P, Karwande SV, Kushner JP, Olsen S, Renlund DG. Successful treatment of a cardiac angiosarcoma with combined modality therapy. J Heart Lung Transplant 1994;13:923–5.[Medline]
- Chachques JC, Argyriadis PG, Latremouille C, D’Attellis N, Fornes P, Bruneval P, et al. Cardiomyoplasty: ventricular reconstruction after tumors resection. J Thorac Cardiovasc Surg 2002;123:889–4.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
