Surgical challenges in the treatment of leiomyosarcoma of the inferior vena cava: analysis of two cases and brief review of the literature

Abstract

Background: Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor of mesenchymal origin. Optimal treatment should include complete resection of the malignant lesion with preservation of venous return. We present our experience from two patients treated in our hospital in the last 3 years.

Methods and results: The first case is that of a 54-year-old woman, with a 9 cm a primary IVC leiomyosarcoma extending from the level of the right renal vein to the common iliac veins. The patient underwent radical tumor resection and reconstruction of the IVC with a polytetrafluoroethylene patch. She received adjuvant chemotherapy and is free of recurrence almost 3 years after surgery. The second case is that of a 56-year-old woman presenting with back pain due to an 8-cm retroperitoneal mass in close proximity to the right renal vein. She underwent exploratory laparotomy, where initially the effort of en bloc resection of the mass failed. Eventually, partial resection of the IVC was performed and the defect was primarily repaired. Pathological examination confirmed primary leiomyosarcoma of the IVC. She received adjuvant chemotherapy, but was referred to our hospital with local recurrence 6 months after the operation and is suffering from disseminated abdominal disease almost a year postsurgery.

Conclusion: Radical surgical en bloc resection is the mainstay of treatment for IVC leiomyosarcomas. Extensive vascular reconstruction techniques may be necessary to restore adequate venous return to the IVC after tumor resection, and combination with adjuvant chemoradiotherapy has been shown to prolong disease-free survival rates.