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      Eleven years' experience with Korean cardiac myxoma patients: focus on embolic complications.

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          Abstract

          Cardiac myxomas are rare but are the most common cardiac tumors. This study is based on our clinical experience with cardiac myxomas over a period of 11 years at Sejong General Hospital. We focused on the embolic complications of patients with cardiac myxoma.

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          The complex of myxomas, spotty pigmentation, and endocrine overactivity.

          Of 40 patients (16 males and 24 females), 29 had cardiac myxoma(s), 14 had skin pigmentation (lentigo and several types of nevi) which also commonly affected the lips, 6 had skin myxoma(s), and 12 had both pigmentation and myxoma(s); 18 had primary pigmented nodular adrenocortical disease (Cushing syndrome was present in 11); 10 had myxoid mammary fibroadenomas; 9 had testicular tumor(s) (large-cell calcifying Sertoli cell tumor, Leydig cell tumor, or adrenocortical rest tumor, or a combination); and 4 had pituitary adenoma with gigantism or acromegaly. The maximum number of conditions present together was five, occurring in two patients; each of the remaining patients had at least two of the conditions. The overlap, in this sizeable number of patients, of various combinations of the same rare or very rare conditions unlikely to occur together by chance with any degree of frequency is striking evidence for a unique syndrome. The patients were young (mean age at diagnosis of the first component, 18 years). Pathologic involvement tended to be multicentric (heart and skin) and bilateral in paired organs (adrenal, breast, and testis). Thirteen patients (32%) are alive and well. Twelve patients are alive but with complications of cardiac myxoma (in 8), testicular tumors (in 2), residual Cushing syndrome (in 1), or bilateral pulmonary nodules (in 1). Twelve patients are dead: 9 of cardiac myxoma, 1 of intracranial (nonpituitary) tumor, and 2 postoperatively. The status of three is unknown.
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            Central nervous system manifestations of cardiac myxoma.

            Neurologic complications can be the initial manifestation of atrial myxoma. Prompt diagnosis is of paramount significance to prevent recurrent complications. To identify patients with neurologic complications attributed to atrial myxoma. With institutional review board approval, we retrospectively reviewed the medical records of 74 consecutive patients with pathologically confirmed cardiac myxoma at the Mayo Clinic from January 1, 1993, through December 31, 2004. Discharge and follow-up modified Rankin score. Nine of the 74 patients with cardiac myxoma (12%) presented with neurologic manifestations in the setting of atrial myxoma. Mean age was 48.5 years (range, 17-70 years). There were 6 females and 3 males. Among patients with myxoma and neurologic symptoms, ischemic cerebral infarct was the most common neurologic manifestation (8 patients [89%]). No patients had concomitant cardiac symptoms. The size of the atrial myxoma was variable, with a mean diameter of 2.7 (range, 0.4-6.5) cm. Most of the atrial myxomas causing neurologic symptoms demonstrated a mobile component on transesophageal echocardiography (8 patients [89%]). Two patients (22%) had pathologic evidence of systemic myxomatous emboli. One patient with intracerebral hemorrhage had pathologically confirmed intracranial metastatic myxoma and myxoma-induced aneurysmal dilatation. Neurologic complications are associated with cardiac myxoma in some patients with myxoma and, when they occur, frequently present with cerebral infarction. The mobility, not the size, of the myxoma appears to be related to embolic potential. Potential delayed neurologic complications relevant to patients with tumor embolization include myxoma-induced cerebral aneurysm and myxomatous metastasis, which can mimic the clinical picture of central nervous system vasculitis or infective endocarditis.
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              Cardiac myxoma: 40 years' experience in 63 patients.

              Cardiac myxomas were resected in 63 patients, including the first successful procedure in the world. Patient data and data obtained during follow-up of the survivors were reviewed. Preoperatively, valve obstruction occurred in 56% and emboli in 32% of the patients. The myxomas were located in the left atrium in 88%, right atrium in 10%, and both atria in 1 patient (1.6%). One patient died early, and 5 died late. The 20-year survival rate was 85%. At a median of 13 years (range, 1 month to 42 years) after resection, 26% of the survivors reported having various cardiopulmonary symptoms, 5% had had thromboembolic events, and 69% were asymptomatic. After 2 years, there was a recurrence in a young woman who had had a multifocal myxoma at the time of the primary operation. No other evidence of recurrence was found at autopsy or during repeat echocardiography in the remaining patients during an observation period of 787 patient-years. Surgical resection of a single myxoma is a safe and effective treatment, with a low risk of recurrence. After uncomplicated resection, the frequency with which postoperative echocardiography is performed should be limited.
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                Author and article information

                Journal
                Cerebrovasc. Dis.
                Cerebrovascular diseases (Basel, Switzerland)
                1421-9786
                1015-9770
                2012
                : 33
                : 5
                Affiliations
                [1 ] Department of Neurology, Sejong General Hospital, Bucheon, Republic of Korea. neurosj@catholic.ac.kr
                Article
                000335830
                10.1159/000335830
                22517375
                262de4d8-75c6-48e0-a762-ebaaf7e26f3f
                Copyright © 2012 S. Karger AG, Basel.
                History

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