Asian Cardiovasc Thorac Ann 2005;13:184-186
© 2005 Asia Publishing EXchange Ltd
Two-Chamber Intracardiac Mesothelioma
Hutan Ashrafian, MBBS,
Thanos Athanasiou, MD,
John Yap, FRCS,
Anthony C DeSouza, FRCS
Department of Cardiothoracic Surgery, St Mary’s & Royal Brompton Hospital, London, United Kingdom
For reprint information contact: Thanos Athanasiou, MD Tel: 44 207 886 6147 Fax: 44 207 886 1762 Email: tathan5253{at}aol.com, Department of Cardiothoracic Surgery, St Mary’s & Royal Brompton Hospital, 70 St Olaf’s Road, London, England SW6 7DN, United Kingdom.
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ABSTRACT
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Primary intracardiac malignant mesotheliomas are extremely rare and carry a very poor prognosis. We present such a case where the lesion encompassed two chambers, the left atrium and ventricle, with no pericardial involvement. Initial echocardiography mimicked a myxoma, and urgent surgical intervention was required in view of significant cardiorespiratory compromise. To the best of our knowledge this is the first case of a primary two-chamber intracardiac malignant sarcomatoid mesothelioma.
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INTRODUCTION
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Primary intracardiac malignant mesothelioma is a rare disease with a difficult diagnosis and poor prognosis. The few case reports to date have only described one chamber cardiac involvement, with no account of a standardized optimal therapy owing to the low incidence of the condition. We present the first case of a two-chamber intracardiac mesothelioma, discussing our experience and approach to its management.
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CASE REPORT
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A 57-year-old female presented to her local emergency department with three days of respiratory insufficiency. History revealed three months of worsening orthopnea, dizzy spells and night sweats. She had no significant past medical history.
A diagnosis of mitral stenosis with gross pulmonary edema was followed up with electrocardiography and chest radiograph, which were unremarkable. Transthoracic echocardiography revealed a left atrial mass attached to the posterior mitral leaflet. Therefore the initial diagnosis of atrial myxoma was considered. Uncontrollable cardiorespiratory compromise resulted in transfer to our centre. Emergency surgical exploration of the heart was performed via sternotomy and accompanied by transesophageal echocardiography (Figure 1
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Figure 1. Transesophageal echocardiogram: four chamber view of the myocardium with a large mass on the surface of the mitral valve in the left atrium. Another small mass is detected on the interventricular septal surface of the left ventricle.
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Bi-caval cardiopulmonary bypass was instituted with 28°C cooling followed by antegrade cardioplegic solution. Intraoperatively no evidence of pericardial involvement was found and therefore the left atrium was approached transseptally. A smooth white non-pedicled left atrial tumor of 2.5 x 3 x 4 cm arose from the posterior wall and extended from the mitral annulus to the origin of the left pulmonary veins. It also extended down into the ventricle with attachment to the papillary muscle and ventricular walls. This tumor was carefully dissected off. This alerted us to the fact that the tumor was unlikely to be a myxoma. Transesophageal echocardiography further confirmed the valve to be competent with good left ventricular function.
Urgent histology of tumor was sent for review by frozen section. Samples from both the left atrium and ventricle revealed clusters of large spindle to pleomorphic cells with large nuclei, prominent nucleoli and mitoses. Paraffin sections revealed a biphasic tumor with epithelial and spindle elements (Figure 2). The tumor stained for calretinin, thrombomodulin and cytokeratins (MNF116, Cam5.2). They did not stain for CK5/6, BerEP4, carcinoembryonic antigen, smooth muscle actin, S100 protein, BCL2, desmin, CD3, CD20, CD34, Factor 8, thyroid transcription factor 1, estrogen receptor, or for mucin with a D-PAS stain. This immunohistochemical profile was in keeping with malignant mesothelioma. With the tumor debulked, the procedure was concluded.
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Figure 2. Photomicrographs: on the left the large malignant cells arranged in clusters which are epithelial. On the right shows malignant spindle cells admixed with inflammatory cells the sarcomatoid area.
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During follow-up no further tumor sites were identified, and at oncological review the possibility of chemotherapy and further surgery was declined by the patient considering the balance of the poor quality of life with treatment and poor likelihood of disease remission. A detailed assessment of asbestosis risk factors revealed possible exposure via her husband who worked as a naval engineer.
A recurrence of the intracardiac lesion progressively increased in size on follow-up magnetic resonance imaging, and the patient developed left and then right cardiac failure over the course of 12 months, which eventually led to death. Postmortem reconfirmed the diagnosis of primary intracardiac two-chamber malignant mesothelioma, which had undergone dedifferentiation and become completely sarcomatous.
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DISCUSSION
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The majority of all cardiac tumors are metastases, with an incidence of primary cardiac tumors at 0.001% to 0.28%.1 Primary malignant mesotheliomas constitute 4% of these, which predominate from the pericardium, accounting for 50% of tumors at this site.1–2 An extremely rare incidence of primary unichamber intracavitary cardiac mesotheliomas correspond to scarce reports in the literature.3 Masses involving the mitral valve can have both a ventricular and an atrial aspect, and this may have existed to some extent with these previous reports. However, this is the first report to date describing an intracardiac mesothelioma extending throughout two chambers in the left heart, running from the left atrium to the papillary muscles.
Three histological subtypes of malignant mesothelioma have been described – epithelial, sarcomatous and mixed. Malignant mesothelioma carries a very poor prognosis with few successful treatment strategies and little benefit from radiotherapy and chemotherapy.4 The course of disease includes thromboembolism, structural heart disease, arrhythmias and metastases. This case demonstrated cellular dedifferentiation with sarcomatous progression and was further complicated by two-chamber involvement, a rare finding amongst primary cardiac neoplasms.5
The surgical approach to such malignant tumors with a poor prognosis includes palliative debulking of mass, with prosthetic valve replacement in cases of unrepairable valvular stenosis or incompetence. Cardiac transplantation is technically possible, allowing complete resection of tumor, and has been performed with varied results in the context of prognostically poor primary malignant tumors of the heart.6
Malignant sarcomatoid intracardiac lesions are extremely infrequent. To the best of our knowledge we present the first case of a primary two-chamber intracardiac malignant sarcomatoid mesothelioma – an exceptionally rare case. Both echocardiography and histopathology are strongly indicated in the management of such intracardiac masses. Surgical aims are to conserve and maintain the structural and physiological integrity of the myocardium so as to maximize any potential benefits of other treatment modalities.
We would like to thank Dr Mary Sheppard for her invaluable help in the preparation of all histopathological material.
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REFERENCES
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