Leiomyosarcoma of the iliac vein is an uncommon tumor. We report a case of a 63-year-old Japanese woman with leiomyosarcoma of the right external iliac vein. The patient complained of right inguinal pain and swelling. Computed tomography demonstrated a mass surrounding the right external iliac artery and vein. Metastases in the lungs and liver were found. Complete resection of the tumor along with the involved vessels was performed. Polytetrafluoroethylene grafts were used to reconstruct the vessels. Pathological examination revealed leiomyosarcoma of the external iliac vein. Although the prognosis of leiomyosarcoma is poor, en bloc tumor resection is the treatment of choice.
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