A case of a thirty-nine-year-old woman with leiomyosarcoma of the inferior vena cava (IVC) is described. The tumor arose from the middle segment of the IVC, and liver metastasis of the tumor was found. The tissue of the tumor obtained at laparotomy was positive for estrogen receptor and progesterone receptor. The patient has been treated with combined multimodal therapy including resection of the main tumor, intrahepato-arterial chemotherapy, and hormonal manipulation. To the authors' knowledge, this is the first case of leiomyosarcoma of the IVC in which the presence of steroid receptors was determined.
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