Leiomyosarcoma of the Inferior Vena Cava: A Multicenter Experience

Objective: Primary leiomyosarcomas of the inferior vena cava (IVC) are best managed with surgical resection when it is technically feasible; however, conduit choice and reconstruction techniques lack consensus. We conducted a comprehensive assessment through the Vascular Low Frequency Disease Consortium to provide a summary of the characteristics of surgical treatment of these rare soft tissue malignant neoplasms. Methods: Multicenter, standardized database review of patients undergoing surgical resection of primary IVC leiomyosarcomas from 2007 to 2017 was conducted. Demographics, periprocedural details, and postoperative outcomes were captured using a RedCap survey and analyzed. Results: A total of 90 patients (64 women and 26 men) with a mean age of 59 years (range, 30-88 years) from 14 institutions were treated. In the entire cohort, preoperative biopsy-proven disease was confirmed in 68%, metastatic disease was present in 23%, and neoadjuvant radiation (31%) or chemotherapy (21%) was employed. Tumor location was distributed as follows: 45 (50%) below the renal veins; 56 (62%) between renal and hepatic veins; and 13 (14%) above the hepatic veins. Surgical resection of other adjacent organs was necessary in 38 (42%) patients, including kidney (n ¼ 23), adrenal (n ¼ 22), bowel (n ¼ 6), liver (n ¼ 5), and ureter (n ¼ 14). Conduits for reconstruction included ringed polytetrafluoroethylene (PTFE; n ¼ 70), nonringed PTFE (n ¼ 1), Dacron (n ¼ 1), autogenous vein (n ¼ 1), bovine pericardium (n ¼ 4), cryopreserved tissue (n ¼ 5), and IVC ligation (n ¼ 3) for caval occlusion. Mean conduit size was 18.5 mm (range, 10-30 mm). An adjunctive arteriovenous fistula to augment flow and to improve patency was done in a minority of cases (n ¼ 5). Complete R0 resection was accomplished in 70 (78%). Pathologic grades were as follows: I, 19%; II, 40%; III, 33%; indeterminate, 8%. In-hospital mortality was 2.2%, with a mean length of stay of 11 days (range, 3- 95 days). Antiplatelet therapy was used in 64% of patients, and longterm anticoagulation was employed in 55% of patients. During a mean follow-up of 35.3 months (range, 0-151 months), mortality was 21.1%, graft occlusion was 8.9%, and in situ tumor recurrence was 12.2%. Conclusions: This multi-institutional study demonstrates that complete en bloc resection of IVC leiomyosarcomas with vascular surgical reconstruction is an effective treatment resulting in low in situ tumor recurrence rates. Ringed PTFE graft is the most commonly used condu