Ann Thorac Surg 1998;66:1791-1792
© 1998 The Society of Thoracic Surgeons
Case Reports
Long-term remission after resection, chemotherapy, and irradiation of a metastatic myxoma
Franziska Bernet, MDa,
Peter M. Stulz, MDa,
Thierry P. Carrel, MDa
a Department of Cardiothoracic Surgery, University Hospital Basel, Basel, Switzerland
Accepted for publication May 1, 1998.
Address reprint requests to Dr Bernet, Clinic for Cardio-Thoracic Surgery, University Hospital, CH-4031 Basel, Switzerland
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Abstract
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Malignant local or metastatic myxomatous tumors of the heart are rare and sometimes present with an unpredictable outcome. The present report demonstrates an unusual case of left atrial myxoma with a rather strange distribution of metastases that had long-term remission after combined surgical resection, chemotherapy, and irradiation of cerebral and pulmonary metastases.
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Introduction
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Primary tumors of the heart are rare; a large proportion is constituted by benign, nonrecurring left atrial myxoma. The histogenesis of the tumor is still unclear [1, 2]. The clinical presentation of cardiac myxoma is variable, ranging from constitutional disorders to hemodynamic symptoms caused by invasion into the left atrium and obstruction of the mitral valve. Although a majority of cardiac myxomas are definitively cured after surgical resection, a small proportion may exhibit a more malignant evolution, characterized by local invasion or metastatic growth of tumor emboli [2, 3].
We present an unusual long-term remission of a malignant myxoma with multiple cerebral, pulmonary, and soft tissue metastases.
A 31-year-old patient was admitted in April 1987 because of fever and exertional dyspnea. He had suffered from a transient ischemic attack with paresis of the left upper extremity before admission.
Transthoracic echocardiography revealed the presence of a left atrial mass that was considered to be the source of cerebral embolism. Surgical exploration was performed through a combined left and right atrial approach soon thereafter. A 4 x 6 x 4-cm pedunculated tumor that originated from the interatrial septum and prolapsed through the mitral valve into the left ventricle was removed with a generous portion of the interatrial septum. Closure of the interatrial septum was realized with a pericardial patch.
The macroscopic aspect of the tumor was polypoid, multilobulate, and friable, typical for a left atrial myxoma. The histologic properties of the tumor differed somewhat from those of a more conventional myxoma: the tumor included a large amount of spindle and polygonal cells with polymorphic and hyperchromic nuclei and demonstrated a high mitotic rate. However, the myxoid stroma and the polypoid-papillary structure gave the tumor an appearance consistent with that of a cardiac myxoma (Fig 1).
The postoperative course was uneventful and the patient was discharged from the hospital 1 week after the operation. Two months later he suffered from general seizure; computed tomography revealed multiple frontal and occipital bilateral cerebral metastases (Fig 2). At this time, transesophageal echocardiography did not demonstrate any tumor recurrence or thrombus formation on the patch.
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Fig 2. Computed tomogram of the brain performed 2 months after surgical excision of the tumor, demonstrating bilateral, frontobasal, and occipital metastases.
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During the same hospitalization, two soft tissue metastases—one located over the scapula and the other in the right gastrocnemius muscle—were resected. Another metastasis was excised from the left gastrocnemius muscle 6 weeks later. The histology of the resected metastases was consistent with that of the primary cardiac tumor (Fig 3). Complete staging of the patient demonstrated a further metastasis located in the lower lobe of the right lung. After irradiation of the cerebral metastases with a total dose of 5,000 Gy and five courses of chemotherapy with doxorubicin and ifosfamide the cerebral and pulmonary metastases disappeared completely. Ten years after the initial resection of the atrial myxoma, the patient still demonstrates complete remission.
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Fig 3. The metastasis within the soft tissue over the scapula (A) and the metastasis in the left gastrocnemius muscle (B) had histologic characteristics similar to those of the primary tumor except for the central tumor necrosis. (x80 before 66% reduction.)
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Comment
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Tumors of the heart, although uncommon, present in protean ways. Whether involving primary or secondary tumors, neoplastic heart disease is expressed in only a limited variety: the triad of obstruction, embolization (sometimes characterized by distant growth of viable embolic material), and constitutional manifestations characterizes intracavitary tumors, especially myxomas [1, 4–6]. Systemic tumor embolization may occur as an initial manifestation or intraoperatively. Therefore, a minimal touch technique is adopted to avoid tumor fragmentation during surgical exploration [7].
The histologic distinction between malignant cardiac myxoma and tumors such as sarcoma with extensive areas of myxoid degeneration may be difficult. In the present case, immunohistologic examination clarified the diagnosis. The tumor demonstrated positive reaction with mesenchymal markers (vimentin, factor VIII) but also with epithelial markers (lu-5, Cam 5.2). The diagnosis of angiosarcoma was excluded because of the absence of reaction with cytokeratin antibodies (coexpression of lu-5 and Cam 5.2) [8].
Our patient demonstrated a malignant clinical evolution with widespreading metastases; however, the histology of the primary tumor was somewhat inconsistent with that of the metastases, which appeared as highly malignant and almost sarcomatous in nature.
According to our own observation, we believe that in young patients with a rather unusual course of their primary intracardiac myxoma, a complete initial staging and a strong follow-up protocol must be instituted to allow early detection of metastatic disease. Long-term remission can be obtained after surgical excision of the tumor and the soft-tissue metastases combined with aggressive chemotherapy and irradiation of cerebral and pulmonary metastases.
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References
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- McAllister HA. Primary tumors and cysts of the heart and pericardium. In Harwey WP, ed. Current problems in cardiology. Chicago: Year Book, 1979;4(2).
- Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-1617.[Free Full Text]
- McCarthy P.M., Piehler J.M., Schaff H.V., et al. The significance of multiple, recurrent and complex cardiac myxoma. J Thorac Cardiovasc Surg 1986;91:389-395.[Abstract]
- Rupp G.M., Heyman R.A., Martinez A.J., Sekhar L.N., Jungreis C.A. The pathology of metastatic cardiac myxoma. Am J Clin Pathol 1989;91:221-227.[Medline]
- Attum A.A., Johnson G.S., Masri Z., Girardet R., Lansing A.M. Malignant clinical behavior of cardiac myxomas and "myxoid imitator". Ann Thorac Surg 1987;44:217-222.[Abstract]
- Read R.C., White H.J., Murphy M.L., et al. The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg 1974;68:857-860.[Medline]
- Cleveland D.C., Westaby S., Karp R.B. Treatment of intra-atrial cardiac tumors. JAMA 1983;249:2799-2805.[Abstract/Free Full Text]
- Curschellas E., Toia D., Borner M., Mihatsch M.J., Gudat F. Cardiac myxomas: immunohistochemical study of benign and malignant variants. Virchows Arch Pathol Anat 1991;418:485-491.
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Abstract
Introduction
