Surgical and reconstructive outcomes in primary venous leiomyosarcoma

Objective: Primary venous leiomyosarcomas (PVL) are rare and pose challenges in surgical management. This study evaluates the clinical outcomes and identifies predictors of survival in our surgical series of PVL. Methods: A retrospective review was performed of patients who had resection of PVL at three centers between 1990 and 2018. Patient demographics, comorbidities, intraoperative data, survival, and graft-related outcomes were recorded. Survival analysis was performed using Kaplan-Meier curves and Cox proportional hazards regression. Results: Seventy patients with a diagnosis of PVL were identified between 1990 and 2018. Fifty-four patients (77%) had PVL of the inferior vena cava (IVC) and 16 (23%) had peripheral PVL. The mean follow-up for the series was 55.0 months (range, 1-217 months). Fifty-one patients (96%) with IVC-PVL needed caval reconstruction and 3 (4%) had resection only. There were no deaths within 30 days of surgery. Five patients (9%) required early reintervention including one (2%) IVC stent. Sixteen peripheral PVL were identified. Eight patients (50%) had venous reconstructions performed and 8 (50%) had the vein resected without reconstruction. There were no deaths within 30 days. Five-year survival was 57.5% for IVC-PVL and 70.0% for peripheral PVL. Kaplan-Meier survival analysis for IVC and peripheral PVL revealed no difference in overall survival (P = .624) at 5 years. Conclusions: PVL is a rare and aggressive disease even with surgical resection. We found no difference in survival between IVC and peripheral lesions, suggesting that aggressive management is warranted for PVL of any origin. Management of PVL requires a multidisciplinary approach to provide patients with the best long-term outcomes.