Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare tumor with poor prognosis. Optimal treatment includes complete resection of the malignant lesion.

From 1997 to 2013, eight patients underwent surgery in our department for IVC LMS. LMS was considered to arise from the IVC if the tumor presented intraluminal development or if complete resection (R0) required removal of part of the IVC with an extraluminal mass.

There were two grade 1 tumors (25%), four grade 2 (50%) and two grade 3 (25%). The median length of stay was 16 days and there were no peri-operative deaths. Median of follow-up was 56 months and mean overall survival was 120 months. Mean 3-year survival rate was 87.5%. Six patients (75%) developed a local recurrence. Four patients died from disease progression. Two patients underwent to surgery for recurrence.

IVC LMS have a poor prognosis if surgical resection cannot be achieved. Long-term survival is related to an extensive surgery, in the event of recurrence, surgery should again be proposed and may be effective for controlling disease progression, possibly improving survival.