Leiomyosarcoma of the Inferior Vena Cava: A Case Series and Review of the Literature.

Background: Leiomyosarcoma of the inferior vena cava (IVC) is an exceedingly rare smooth muscle sarcoma. Approximately 300 cases have been described in the literature, and further research is needed to understand the disease and guide its management. Surgery remains the only potential curative measure.

Methods: A retrospective chart review of patients who underwent surgical resection of IVC leiomyosarcoma at our institution over the past 3 years was performed. The patients were identified using a prospectively maintained database.

Results: Three patients with leiomyosarcoma of the infrahepatic IVC underwent radical resection carried out by a team of surgical oncologists and vascular surgeons. There were 2 males (66.7%) and 1 female (33.3%). Mean age at diagnosis was 60.3 years (range 43-78). Mean tumor size was 12.2 cm (range 5.6-22). The mean operative time was 320 min (range 180-421), mean estimated blood loss was 1,300 mL (100-2,000) mL, and average length of stay 8.67 days (6-12). All patients achieved grossly negative margins (R1 or R0 resections) and are alive with a mean overall survival of 21 months (range 12-30). Patient 1 was a 60-year-old man who presented with metachronous skin leiomyosarcomas at 2 different sites. He underwent PET/CT scan that revealed an IVC mass. Resection of the middle segment of the IVC and right kidney was performed with reconstruction with polytetrafluorethylene (PTFE) graft. Patient 2 was a 78-year-old man with an incidentally found a 9-cm IVC tumor. Resection of the tumor was performed, and no reconstruction was needed since the tumor had a completely extraluminal growth pattern. Patient 3 was a 43-year-old woman who presented with abdominal pain. Her work-up showed a 15-cm IVC mass. She underwent resection of the middle segment of the IVC, right nephrectomy, and cholecystectomy with reconstruction of the IVC with PTFE graft.

Conclusions: Surgical resection is the mainstay of treatment in patients with leiomyosarcoma of the IVC. A collaborative approach involving surgical oncologists and vascular surgeons ensures adequate resection with functional reconstruction to achieve the best patient outcomes.