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

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.