Intravenous leiomyomatosis with inferior vena cava and heart extension

Background: Intravenous leiomyomatosis (IVL) is a rare tumor that is histologically benign but biologically malignant. Less than 200 cases of IVL have been reported, most of them were individual reports. Methods: Six patients with IVL involving the inferior vena cava were analyzed. Results: Three patients received one-stage operations and two received two-stage operations. All operations were successful. No perioperative death or other complications were observed. Primary tumors and intravenous tumorous emboli were completely resected from four patients. Residual tumor remained in one patient who had serious adhesions due to multiple previous surgeries; however, with antiestrogen therapy, the residual tumor significantly regressed. All patients had tumor relapse after the operation. Conclusions: IVL should be given more attention by vascular surgeons, although it is extremely rare. Many therapeutic methods are available for uterine leiomyomatosis involving inferior vena cava, among which operation is the best choice. (J Vasc Surg 2009;50:897-902.)