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
