Leiomyosarcoma of the inferior vena cava: case report and treatment of recurrence with repeat surgery.

Leiomyosarcoma of the inferior vena cava (IVC) is an extremely rare malignancy with poor prognosis due to late diagnosis. Surgical resection currently remains the best treatment; however, recurrence frequently occurs and the 5-year survival rate is only 31%. The aim of this study is to report a case of IVC leiomyosarcoma and treatment of recurrence with repeat surgery. A 36-year-old woman with a high-grade leiomyosarcoma originating from the infrahepatic IVC underwent an en bloc excision of the tumor. Eleven months after the initial operation, two metastases to the omentum were observed. Since the patient showed no response to adjuvant chemotherapy (i.e., a combination of 5-fluorouracil and gemcitabine), repeat operations were used as the main treatment modality for recurrence. The median time to recurrence was 15 months (range 8-27). The middle and upper IVC segments were involved in the local recurrence, and metastatic lesions occurred in multiple sites including the stomach, omentum, mesentery, left liver, and pelvic cavity. Repeat operations to remove the recurrent and metastatic tumors led to a long-term (at least 7 years) survival, and the patient is still alive. Postoperative recoveries were uneventful. Neither complication related to the venous blood flow in the IVC nor renal impairment was noted. Our results suggest that in the setting of chemotherapy-refractory IVC leiomyosarcoma repeat surgery may be an alternative treatment for recurrence and improve survival time. Copyright (c) 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.