Surgical Outcomes of Patients with Inferior Vena Cava Leiomyosarcoma.

INTRODUCTION:Primary vascular leiomyosarcomas are incredibly rare and have poor prognoses. The purpose of this study was to analyze surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma. METHODS:Retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, which included 10 females and 3 males. Presenting symptoms, tumor characteristics, operative management, post-operative complications, and survival rates were assessed for each patient. RESULTS:The median patient age was 59 years old (Q1:52, Q3:68). The median tumor size was 7.0 cm (Q1:6, Q3:12). The median mitotic rate was 6 per 10 HPF (Q1:2.5, Q3:15.5). All 13 patients had grossly negative tumor resections, with 9 (69%) having microscopically negative margins (R0). No patients had lymph node involvement. The IVCs were managed with ligation in 4 cases for tumors already occluding the IVC, and bovine pericardial patch angioplasty in 7 cases, or primary repair in 2 cases for patent IVCs. Concomitant right nephrectomy was performed in 7 patients. Left renal vein ligation was performed in three patients, but no left nephrectomies were performed. Significant postoperative complications included one patient with lower extremity compartment syndrome, two patients with severe leg swelling, and one patient with arm swelling. The 30-day mortality rate was zero. Using the Kaplan-Meier product limit method, disease-specific survival was estimated to be 93%. CONCLUSIONS:Surgical resection is a feasible and effective oncologic treatment option for patients with IVC leiomyosarcomas. The IVC can be safely managed by ligation, primary repair, or patch angioplasty dependent upon the prior patency of the IVC.