Ann Thorac Surg 2001;72:623-625
© 2001 The Society of Thoracic Surgeons
Case report
Metastatic cardiac myxoma
Kamil Kaynak, MDa,
Kazim Besirli, MDa,
Caner Arslan, MDa,
Mustafa Özgüroglu, MDb,
Büge Öz, MDc
a Department of Thoracic and Cardiovascular Surgery, Cerrahpa
a Faculty of Medicine, University of Istanbul, Istanbul, Turkey
b Department of Medical Oncology, Cerrahpaa Faculty of Medicine, University of Istanbul, Istanbul, Turkey
c Department of Pathology, Cerrahpaa Faculty of Medicine, University of Istanbul, Istanbul, Turkey
Accepted for publication August 17, 2000.
Address reprint requests to Dr Kaynak, I. Ü. Cerrahpasa Tip Fakültesi, Gö
üs Kalp ve Damar Cerrahisi Anabilim Dali, 34300, Aksaray/Istanbul, Turkey
e-mail: kamil{at}istanbul.edu.tr
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Abstract
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A 22-year-old woman who had a history of three cardiac operations and a bilateral femoral embolectomy for recurrent cardiac myxoma and myxoma embolism, respectively, was accepted to our clinic with multiple immobile peripheral masses. One of them was compressing the left common femoral artery. This mass was extirpated. Pathology examination revealed myxoma. Chemotherapy was given to the patient and regression of the masses was observed.
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Introduction
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A 22-year-old woman with multiple peripheral masses was referred to our clinic from another hospital in the same city. She had the history of three open heart operations and bilateral femoral embolectomy for cardiac myxoma. One of these peripheral masses extirpated showed the same pathologic features as cardiac myxomas previously resected. Chemotherapy was tried for the other peripheral masses.
Twenty-two months earlier she had developed symptoms of pulmonary venous hypertension such as dyspnea, and hemoptysis. Echocardiography showed a left atrial myxoma filling the left atrial cavity and slight mitral valve prolapse (MPV). She also had anemia and thrombocytopenia. Under cardiopulmonary bypass (CPB), the mass was extirpated with its pedicle. Its pathology examination revealed myxoma showing mitotic activation, necrosis and pleomorphism atypical for classic myxoma.
Six months later she was accepted to the same hospital with sudden onset of numbness, pallor, pain, and coldness of lower extremities. There was no pulse bilaterally at the popliteal and more distal arteries. On echocardiography, a large left ventricular myxoma was detected. Emergency bilateral femoral embolectomy was performed and a large amount of myxomatous material was taken out from the lower extremity arteries. In the same operation, CPB was undertaken, and with left ventriculotomy, the mass was extirpated by cauterizing its pedicle from the interventricular septum. Pathology examination results were the same as before.
Four months after the second operation the patient had a syncopal attack and echocardiography showed that the left ventricle was filled with a tumor extending to the ascending aorta. Under CPB, left ventriculotomy and aortotomy were undertaken and myxoma was extirpated with its pedicle and a mass of muscle from the interventricular septum and this area cauterized. Pathology results of this mass were the same as before.
Four months ago, pain developed in her left groin and she found a mass there on self-examination. In the following 3 weeks, new immobile masses ranging from 1.5 cm to 9 cm in size appeared on her left thigh, left buttock, on the dorsum of the right foot, and the right wrist (Fig 1). Routine blood and urine analysis were normal. Magnetic resonance imaging showed that intensities of these soft tissue masses correlating with that of myxoma (Fig 2).
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Fig 2. Magnetic resonance imaging demonstrates myxoma masses in the left gluteal region and left thigh posteriorly surrounding the femur.
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The mass in the left inguinal region was extirpated with a small segment of the common femoral artery. Pathologic examination of this mass revealed myxoma showing malign features such as pleomorphism, prominent mitotic activation, and perineural invasion (Fig 3). We decided to begin chemotherapy for the other masses. A drug regimen composed of doxorubicin 20 mg/m2, ifosfamid 2 g/m2, and mesna 2 g/m2 was undertaken for 2 days every month. Regression of the masses was observed after 3 months (Fig 4).
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Fig 3. Photomicrograph of the left femoral myxoma extirpated surgically shows histologic features of malignant myxoma. Note the pleomorphism and hypercellularity with occasional tumoral giant cells (hematoxylin and eosin, x 100).
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Comment
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Cardiac myxomas usually present as benign pedunculated neoplasms arising from the atrial septum in the region of the fossa ovalis. Approximately 80% occur in the left atrium and may cause intermittent mitral valvular obstruction. Resection is straightforward. However, these neoplasms may show malignant behavior. Dang and Hurley [1] pointed out that malignancy of atrial myxomas is predicated on biologic behavior rather than on histologic appearance. Read and coworkers [2] described a case of metastatic myxoma to soft tissue and bone. Cerebral metastasis from myxoma has also been reported [3]. Patrick and associates [4] reported that patients at high risk for multiple metachronous myxomas might be identified preoperatively by the presence of a positive family history of cardiac myxoma or elements of the myxoma complex.
Our patient has no component of a myxoma complex such as lentigines, pituitary, adrenocortical, or breast masses (fibroadenoma) [4]. She also has no family history of this lesion. Atrial septum resection was not performed in the first operation in the light of previous pathology. In the first and second recurrences, tumors were aggressively excised from the left ventricle. Although no cardiac recurrence was seen, multiple peripheric myxomas had grown rapidly in a 3-week period, 7 months after the operation performed for a second cardiac recurrence. We concluded that recurrent myxoma filling the left ventricle had caused multiple peripheral tumoral microemboli or metastases at any time during the course of disease. Although the roles of chemotherapy and radiation therapy in the treatment of recurrent or metastatic myxoma remain undefined, we applied chemotherapy and saw regression of these masses in 3 months. We want to emphasize that chemotherapy may be a modality to control metastatic myxomas when surgery is contraindicated.
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References
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Dang C.R., Hurley E.J. Contralateral recurrent myxoma of the heart. Ann Thorac Surg 1976;21:59-62.[Abstract/Free Full Text]
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Read R.C., White H.J., Muphy M.L., Willams D., Sun S.N., Flanagan W.H. The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg 1974;68:857-867.[Medline]
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New P.J.F., Price D.L., Carter B. Cerebral angiography in cardiac myxoma: correlation of angiographic and histopathological findings. Radiology 1970;96:335-345.[Abstract/Free Full Text]
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Patrick M.M.C., Jeffry M.P., Hartzell V.S., et al. The significance of multiple, recurrent and complex myxomas. J Thorac Cardiovasc Surg 1986;91:389-396.[Abstract]
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Abstract
Introduction
