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 surgical and adjuvant treatment strategies are not well defined.
Methods: We performed a retrospective review of eight patients with IVC leiomyosarcoma. We evaluated clinical presentations, operative techniques, patterns of recurrence and survival.
Results: From 1990 to 2007, 8 patients (4 females) underwent curative resection. Median age was 58 years (40-76). Presentations included abdominal pain (5), back pain (2), leg swelling (3) and abdominal mass (2). Preoperative CT or MRI scans showed location of the tumor to be from the right atrium to renal veins (1), retrohepatic (4), and from hepatic veins to the iliac bifurcations (3). En bloc resection included right nephrectomy (3), right adrenalectomy (4), pancreaticoduodenectomy (1), right hepatic trisegmentectomy (1) and right hemicolectomy (1). The IVC was ligated in 5 patients, and a prosthetic graft was used for IVC reconstruction in 3 patients. Median size of the tumor was 10 cm in diameter (6-30). Resection margins were negative in 6 cases and microscopically positive in 2 cases. Median length of stay was 13 days (6-22). Morbidity included two cases of atrial fibrillation, one retroperitoneal hematoma, and one transient hepatic encephalopathy. There was one postoperative death from multi-system organ failure. Three patients received adjuvant chemotherapy and/or radiation. Median follow-up was 43 months (2-111). 7 patients were alive at last follow-up. 4 patients had recurrence; liver (3), retroperitoneum (2) and lungs (1). The median time interval to recurrence was 14 months (3-28). 2 patients underwent successful resection of recurrence (ex-vivo right hepatic lobectomy and resection of a retroperitoneal mass respectively), and one received TACE for intrahepatic 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 local recurrence should be considered.