Eur J Cardiothorac Surg 2002;21:124-126
© 2002 Elsevier Science NL
Primary malignant mesenchymoma of the heart
Ali Kutsala,
Selim Tansala,
Hüseyin Okutana*,
lhan Tuncerb
a Department of Thoracic and Cardiovascular Surgery, Süleyman Demirel University Medical School, Isparta, Turkey
b Department of Pathology, Çukurova University Medical School, Adana, Turkey
Received 18 June 2001;
received in revised form 10 October 2001;
accepted 10 October 2001.
* Corresponding author. Tel.: +90-246-2326657; fax: +90-246-2324510
e-mail: okutanh{at}yahoo.com
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Abstract
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Primary malignant cardiac tumours are uncommon, and cardiac malignant mesenchymoma is extremely rare. A case of primary malignant mesenchymoma in a 41-year-old woman arousing from the left atrial septum, obstructing the mitral orifice by passing through it into the left ventricle is described. The tumour was fully resected, and adjuvant chemotherapy was applied, but the patient had died by tumour recurrence in 8 months.
Key Words: Mesenchymoma • Heart neoplasms • Mesenchymoma surgery • Cardiac tumours • Heart surgery
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1. Introduction
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Primary tumours of the heart and pericardium are rare, occurring with a frequency of 0.001–0.28% in reported or collected postmortem series of which 75% are benign, and 25% are malign in origin [1].
Malignant mesenchymoma is a rare soft tissue neoplasm showing at least two types of malignant mesenchymal differentiation in addition to poorly differentiated fibrosarcomatous element [2]. Since Stout's original series of eight cases and a further series in children only isolated case reports have appeared in the literature [3].
A case of a malignant mesenchymoma of the heart presenting itself as mitral stenosis, and treated by resection will be discussed in the monograph.
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2. Case report
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A 41-year-old woman was admitted to our clinic with severe palpitation and dyspnea. On physical examination her heart rate was 132 per min, and the systemic blood pressure was 95/65 mmHg. A moderate diastolic murmur was heard at the apex.
The chest roentgenogram showed a cardiothoracic ratio of 65%. Echocardiographic examination revealed a huge mass localised mostly in the left atrium and passing through the mitral valve into left ventricle.
On 16th August 1997 the operation was performed under cardiopulmonary bypass. The left atrium was opened. There was a 5x3x3.5 cm solid mass originating from the atrial septum, passing through the mitral valve into the left ventricle causing mitral obstruction (Fig. 1). The tumour was fully resected with its attachment, and the defect at the atrial septum was closed primarily.
The histopathological examination revealed that the tumour was composed of multible mesenchymal elements of chondrosarcoma, leiomyosarcoma and rhabdomyosarcoma, and was diagnosed as malignant mesenchymoma (Fig. 2).
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Fig. 2. Histopathologic examination of resected mass from the patient. Areas of malign cartilagenous cells (chondrosarcoma) and myosarcomatous cells (leiomyosarcoma) with muscle of heart. (haematoxylin Eosin stain, original magnification x200).
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The postoperative course was uneventful, and the patient was discharged from the hospital on the 7th postoperative day. Adjuvant chemotherapy was applied in an Oncology Hospital, but after 8 months she was admitted again with severe dyspnea and congestive heart failure. The echocardiographic examination revealed a large mass in the same localisation The patient had died due to recurrent mechanical obstruction of the mitral valve before the second urgent operation.
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3. Discussion
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Primary cardiac tumours are rare pathologies 75% of which are benign, and 25% are malign in origin, and they are up to 20 times less frequent than are secondary tumours of the heart. The prevalence of primary cardiac tumours other than benign myxomas is of course even lower. In 52,500 patients who underwent cardiac operations during a 22-year period, Reece found only 71 cases of primary tumours, 51 of which were myxomas [4]. In a another series, of 4769 patients autopsied were found 1148 males (%42) and 780 female (%38) had a primary tumour. Among them, 162 (8.4%) subjects had cardiac metastases [5].
The great majority of the malignant ones are sarcomas, most frequently angiosarcomas. Rhabdomyosarcoma, fibrosarcoma, liposarcoma, primary malignant lymphoma, and occasional sarcomas of other basic cell types constitute the remaining, but infrequent primary malignant cardiac tumours [4,6]. Primary malignant mesenchymoma involving the heart is extremely rare. There were reported only 19 manuscript about the subject in English literature until 1999. It is generally regarded as a high-grade tumour [7,8].
Depending on the size and location of the tumour, it may produce a wide variety of signs and symptoms, such as chest pain, heart murmurs, arryhtmias, signs of congestive heart failure or embolisation and cardiac enlargement [8].
Echocardiography is the essential method of detecting cardiac tumours. Transthoracic and transoesophegeal echocardiography enables determination of location and size [9,10]. Ultrafast computed tomography provides precise spatial localisation of intracardiac masses. High resolution is similarly achieved by magnetic resonance imaging, which is demonstrating increased usefulness in detection and characterisation of intracardiac masses. Gated MRI is useful for the assessment of cardiac masses, since it provides fine spatial and contrast resolution [1,2].
Although the diagnosis of cardiac masses is easily attainable by routine imaging techniques, differential diagnosis between primary and secondary tumours, malignant and benign forms, and non-neoplastic masses, is achievable only by a thorough microscopic study of surgical resections [6].
Frandsen and colleagues had reported a case of malignant mesenchymoma of the left atrium obstructing the mitral orifice, which was revealed at autopsy in a 39-year-old woman [11].
Peters et al. have reported a patient presenting with a history and clinical findings typical of mitral stenosis caused by two tumoral masses: one on the posterior mitral leaflet causing mitral obstruction, the other arising in the region of the lower pulmonary vein orifice and obstruction inflow through the vein [9].
It is generally thought that malignant mesenchymoma is a highly malignant neoplasm with a poor prognosis. Although Newman and colleagues [3] were disagree in regard to prognosis, and had found that only one of their nine neoplasms led to the death of the patient. They were reported that in contrast to most current opinion, these data suggest that malignant mesenchymoma is not as aggressive as the histology would imply. However, despite the full resection of the tumour, and the chemotherapy our patient had died with the tumour recurrence in 8 months.
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Footnotes
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Presented at the National Symposium on Oncology, Istanbul, Turkey, 30 April–4 May 2001.
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References
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Holley D.G., Martin G.R., Brenner J.I., Fyfe D.A., Huhta J.C., Kleinman C.S., Ritter S.B., Silverman N.H. Diagnosis and management of fetal cardiac tumours: A multicenter experience and review of published reports. J Am Coll Cardiol 1995:516-520.
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Brennan F.M., Casper S.E., Harrison B.L. Sarcomas of the soft tissue and bone. In: DeVita T.V., Hellman S., Rosenberg A.S., eds. The textbook of cancer, principles and practice of oncology, 5th ed Philadelphia: Lippincott-Raven Co, 1997:1752.
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Newman P.L., Fletcher C.D.M. Malignant mesenchymoma. Clinicopathologic analysis of a series with evidence of low-grade behaviour. Am J Surg Pathol 1991;15:607-614.[Medline]
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Reece I.J., Cooley D.A., Frazier O.H., Hallman G.L., Powers P.L., Montero C.G. Cardiac tumours: clinical spectrum and prognosis of lesion other than classical benign myxoma in 20 patients. J Thorac Cardiovasc Surg 1984;88:439-446.[Abstract]
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McKenney P.A., Moroz K., Haudenschield C.C., Shemin R.J., Davidoff R. Malignant mesenchymoma as a primary cardiac tumour. Am Heart J 1992;123:1071-1075.[Medline]
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Peters P., Flachskampf F.A., Haumtmann S., Lo H.B., Schuster C.J. Bilocular atrial malignant mesenchymoma causing mitral and localized pulmonary vein flow obstruction: diagnosis by transoesophageal echocardiography. Eur Heart J 1992;13:1585-1588.[Abstract/Free Full Text]
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DeVille J.B., Corley D., Jin B.S., de Castro C.M., Hall R.J., Wilansky S. Assessment of intracardiac masses by transoesephageal echocardiography. Tex Heart Inst J 1995;22:134-137.[Medline]
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Frandsen N.E., Andersen G., Nielsen J.R. Malignant mesenchymoma of the heart presenting as mitral stenosis. Acta Med Scand 1981;209:235-237.[Medline]
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Abstract
1. Introduction
