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This is a detailed clinical and autopsy documentation of a rare entity--primary cardiac myxosarcoma in a 29-month-old girl. The patient had sudden onset of right hemiplegia and angiographic evidence of multiple occlusions of the left middle cerebral artery. Subsequent M-mode and sector echocardiography showed a mobile, pedunculated left atrial tumor, which was excised. No other tumor mass was noted at the time of surgical exploration, and postoperatively, the patient received a course of chemotherapy (vincristine, dactinomycin, and cyclophosphamide). After a temporary improvement in her condition, the patient died following several days of rapid deterioration; this was 3 months after the onset of symptoms. Autopsy showed that death was due to brainstem herniation secondary to massive infiltration of the brain by tumor, and there were also widespread systemic metastases.
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