Surgical management of leiomyosarcoma of the inferior vena cava.

INTRODUCTION: Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor for which en bloc resection offers the only chance of cure. Due to its rarity, however, optimal strategies for the management of the primary tumor and subsequent recurrences are not well defined.
METHODS: We performed a retrospective review of patients who underwent surgical resection of IVC leiomyosarcoma. We evaluated clinical presentations, operative techniques, patterns of recurrence and survival.
RESULTS: From 1990 to 2008, nine patients (four females) were identified. Median age was 55 years (40-76). Presentations included abdominal pain (n = 5), back pain (n = 2), leg swelling (n = 4) and abdominal mass (n = 2). Pre-operative imaging studies showed tumor location to be from the right atrium to renal veins (n = 1), retrohepatic (n = 5), and from hepatic veins to the iliac bifurcations (n = 3). En bloc resection included right nephrectomy (n = 5), right adrenalectomy (n = 4), pancreaticoduodenectomy (n = 1), right hepatic trisectionectomy (n = 1) and right hemicolectomy (n = 1). The IVC was ligated in six patients, and a prosthetic graft was used for IVC reconstruction in three patients. Resection margins were negative in seven cases. Median length of stay was 12 days (range, 6-22 days). Major morbidity included renal failure (n = 1) and there was one post-operative mortality. Five patients had leg edema post-operatively, four of whom had IVC ligation. Median survival was 47 months (range, 1-181 months). Four patients had recurrence and the median time to recurrence was 14 months (range, 3-25 months). Two patients underwent successful resection of recurrence.
CONCLUSIONS: Curative resection of IVC leiomyosarcoma can lead to long-term survival. However, recurrence is common, and effective adjuvant treatments are needed. In selected cases, aggressive surgical treatment of recurrence should be considered.