Intravenous leiomyomatosis (IVL) with cardiac extension is a rare uterine tumour. We present an unusual case of uterine leiomyoma that progressed along the inferior vena cava into the right atrium. Complete one stage removal of the tumour was performed using cardiopulmonary bypass and circulatory arrest. The literature review reveals that this is the first reported case in Australia of IVL with intracardiac extension which was successfully removed with a single stage procedure.
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