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Primary Venous Leiomyosarcoma: reconstructive and oncologic outcomes
William W. Sheaffer, MD1, Victor J. Davila, MD1, Bernardo C. Mendes, MD2, Andrew J. Meltzer, MD1, William M. Stone, MD1, Samuel R. Money, MD1, Thomas C. Bower, MD2.
1Mayo Clinic in Arizona, Phoenix, AZ, USA, 2Mayo Clinic, Rochester, MN, USA.

OBJECTIVES: Primary venous leimyosarcomas (PVL) are rare and pose challenges in oncologic management. Surgical resection is the primary therapy with less clear roles for chemotherapy and radiation. 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 1990-2018. Patient demographics, comorbidities, tumor characteristics, intraoperative data, oncologic 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 IVC and 16 (23%) had peripheral PVL. 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. Seventeen patients (31%) received neoadjuvant radiation, 6 (11%) neoadjuvant chemotherapy, and 3 (6%) patients received both. There were no deaths within thirty days of surgery. Five patients (9%) required early re-intervention including one (2%) IVC stent. Thirty-one patients (57%) developed metastatic disease and 11 (20%) of these patients developed local recurrence. 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 thirty days. Nine patients (56%) developed metastasis and 3 (19%) developed local recurrence. 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 = 0.624) and tumor free survival (p = 0.644) at 5 years. Cox proportional hazards regression analysis for overall survival revealed neoadjuvant radiation and need for venous reconstruction to be associated with decreased survival (Table 1). CONCLUSIONS:
PVL is a rare and aggressive disease even with surgical resection. We found no difference in survival between IVC and peripheral lesions suggesting aggressive management is warranted for PVL of any origin. IVC resection and replacement can be done safely if needed. The role for adjuvant therapies remains undefined.

Cox regression for overall survival
ParameterHazards Ratio (95% CI)P-value
Age > 701.09 (0.4-2.6)0.86
Gender0.58 (0.2-1.4)0.23
IVC vs peripheral0.58 (0.1-2.6)0.48
Neoadjuvant chemotherapy0.76(0.2-2.5)0.65
Neoadjuvant radiation8.08 (1.8-37.3)0.01
Intraoperative radiation0.60 (0.2-1.9)0.38
Venous resection only0.26 (0.1-0.7)0.005
High grade1.3 (0.4-3.8)0.62


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