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J Thorac Cardiovasc Surg 2002;124:392-394
© 2002 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Cardiothoracic Surgerya and Histopathology,b Leeds General Infirmary, and the Cytogenetics Unit,c St James Hospital, Leeds, United Kingdom.
Received for publication June 8, 2001. Accepted for publication Aug 11, 2001. Address for reprints: R. Unnikrishnan Nair, FRCS, Department of Cardiothoracic Surgery, Leeds General Infirmary, Leeds LS1 3EX, United Kingdom.
Primary cardiac tumors are rare
1 and about 75% are benign, of which 75% are atrial myxomas. 2 Angiosarcomas are the most common malignant primary cardiac tumors, with 90% arising in the left atrium and 10% in the right atrium. 3 This is the first report of a primary cardiac tumor, an angiosarcoma, with a familial incidence.
Clinical Summaries
Case 1
A previously healthy 24-year-old woman sought treatment with chest pain, exertional dyspnea, and episodic dizziness. A transthoracic echocardiogram revealed an 8.9 x 8.4-cm right atrial mass, which was confirmed by computed tomography and magnetic resonance imaging. This proved to be angiosarcoma (Figure 1). There were no distant metastases. Surgery was aimed at debulking the tumor to alleviate obstructive symptoms. At operation, the right atrial free wall was excised and reconstructed with a bovine pericardial patch (Baxter Healthcare Corporation CardioVascular Group, Irvine, Calif), and the right ventricle was debulked. Adjuvant chemotherapy was given. Four months after the operation the patient was well, but at 6 months pulmonary metastases developed. The patient died 9 months after the operation.
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The cardiac tumors in cases 1 and 2 were histologically similar, composed of pleomorphic spindle-shaped and epithelioid cells with high nuclear cytoplasmic ratios and eosinophilic cytoplasm (Figure 2). In places these cells lined anastomosing vascular spaces. Both tumors were immunopositive for endothelial antigens CD31 and CD34. These features indicate that both tumors were angiosarcomas.
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4 Culture was established in flasks containing a 50:50 mixture of Ham F10 medium buffered with N-2-hydroxyethylpiperazine-N-2-ethanesulfonic acid and Roswell Park Memorial Institute medium buffered with bicarbonate, supplemented with 10% fetal calf serum, along with antibiotics and glutamine. Cells were grown in an open system for 6 days and harvested according to standard techniques. Slides were analyzed by Giemsa/Trypsin/Leishman banding. Although there was some variation from cell to cell, consistent clonal chromosome changes are described in the following composite karyotype (Figure 3): 50-51, XX,+1[5],der(1;13)(ql0;ql0)[3],+2[5],+7[5],+8[5], add(8)(p2?)x2[6], + add(8)(p23)[2],+18[3],-19[4],+22[4][cp6].
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Comment
Primary tumors have been recognized as rare cardiac diseases since Columbus of Padua's description in 1559. The first antemortem diagnosis was recorded in 1934. The introduction of echocardiography in 1968 revolutionized the diagnosis of these tumors, allowing their preoperative localization. Magnetic resonance imaging can define the extent of invasion and may allow histogenetic classification.
Most malignant cardiac tumors are sarcomas. 5 Angiosarcomas are the most common sarcomas, 3 accounting for 26% to 37%. They commonly occur in the right atrium, as do other malignant tumors. Right atrial tumors, as in our case 1, are more likely to be malignant than are left atrial tumors. 6 As many as half of all right atrial tumors are malignant. Twice as many men as women are affected, in the opposite ratio to atrial myxomas.
There is little cytogenetic documentation of angiosarcomas, 7 and at present no specific abnormalities are recognized. Our case 1 demonstrated a hyperdiploid karyotype with trisomy of chromosomes 1, 2, 7, 8, 18, and 22 and monosomy of chromosome 19. Of particular interest was the presence of two copies (occasionally three) of an unbalanced rearrangement of 8p. This appears to have resulted in the loss of 8p2 to 8pter, a region frequently deleted in solid tumors. Such homozygous loss suggests the possible involvement of a tumor suppressor gene in this region. A number of candidate genes on 8p have been described in other tumor types. These include DLC1, a suggested candidate tumor suppressor gene at 8p22-8p21.3 implicated in liver, prostate, lung, colorectal, and breast cancers 8; N33, a gene within a homozygous 8p22 deletion seen in prostate cancer 9; and PDGRL at 8p22-8p21.3, which is involved in hepatocellular cancer. 10 We therefore recommend further investigation of 8p in angiosarcomas, with particular regard to any possible loss of heterozygosity, to establish whether this region plays a role in the pathogenesis of this tumor type.
Cardiac angiosarcomas usually have symptoms caused by four pathophysiologic mechanisms but may also be found incidentally. Obstruction of intracardiac blood flow or valves and caval obstruction can also occur. Local invasion may lead to arrhythmias, pericardial effusion or tamponade, and sudden death from arrhythmias or embolization. Eighty percent of patients have systemic metastasis at the time of diagnosis. The most common presentation is congestive heart failure (75%), followed by embolism, tachyarrhythmia, and chest pain. 2 Our patients had three of these features. Cardiac tumors may cause systemic or constitutional symptoms, such as malaise and fever.
Wide surgical excision remains the cornerstone of surgical management of cardiac tumors. It may be difficult to obtain tumor-free margins because of local spread or distant metastases. Complete resection is possible in as many as a third of cases. 6 A few patients with cardiac sarcomas have received transplants, although the role of cardiac transplantation remains unclear. 11,12 If resection is incomplete, the tumor may recur even after chemotherapy and the patient may die of metastatic disease. The long-term results are poor, with an overall survival of less than 1 year. With unresectable sarcomas, radiation and chemotherapy with adriamycin give a limited response.
This report describes a cardiac hemangiosarcoma in a 24-year-old woman whose father had died at the age of 31 years of a histologically and immunochemically identical tumor. We believe that this is the first reported description in the literature of familial angiosarcoma of the heart.
References
This article has been cited by other articles:
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  I. M. Keeling, F. Ploner, and B. Rigler Familial cardiac angiosarcoma. Ann. Thorac. Surg., October 1, 2006; 82(4): 1576 - 1576. [Full Text] [PDF]
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 M. Okada, E. Takeuchi, Y. Mori, S. Ichihara, A. Usui, and Y. Ueda An autopsy case of angiosarcoma arising around a woven Dacron prosthesis after a Cabrol operation J. Thorac. Cardiovasc. Surg., June 1, 2004; 127(6): 1843 - 1845. [Full Text] [PDF]
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