Primary leiomyosarcoma of the inferior vena cava: a 2-institution analysis of outcomes

Abstract

Background: Approximately 300 cases of leiomyosarcoma of the inferior vena cava (IVC) have been reported in the literature to date. In this study, we combined the experience from 2 institutions to provide additional clinical outcomes data.

Methods: We performed a retrospective analysis from 1984 to 2009 that included 17 patients treated between the 2 institutions. Clinicopathologic data, surgical and adjuvant therapy, and survival outcomes were obtained.

Results: The median age of patients in the study was 48 years. The tumor location was infrarenal in 8 patients, juxtarenal in 6, and suprahepatic in 2 patients; 7 patients had high-grade tumors. All patients underwent complete resection; the IVC was repaired primarily in 5 patients, ligated in 5, and reconstructed with a prosthetic tube graft in 7 patients. There was no perioperative mortality; 6 patients had complications. Median follow-up was 49 months; median survival had not been reached when this paper was written. The 5-year overall and disease -free survival were 56% and 37%, respectively. Of the 17 patients, 10 experienced disease recurrence and underwent numerous treatment modalities for these recurrences.

Conclusion: Aggressive resection of primary leiomyosarcoma of the IVC can be performed safely and result in long-term survival, irrespective of IVC management. Despite high recurrence rates, no consensus yet exists regarding adjuvant treatment.