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Intravenous leiomyomatosis is a rare tumor in which benign smooth muscle cells grow into the pelvic venous channels of female patients. A case of intravenous leiomyomatosis with cardiac extension in a 45-year-old woman is described. The patient was diagnosed with cardiac syncope 3 months after total abdominal hysterectomy and was successfully treated with a two-stage approach consisting of sternotomy followed by laparotomy. The cause, disease, presentation, diagnosis, treatment and recurrence are reviewed.

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