Abstract：
BACKGROUND: Primary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma.
METHODS: We performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient.
RESULTS: The median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven 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 leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC.
METHODS: We performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient.
RESULTS: The median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven 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 leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC.
摘要：
背景：原发性血管平滑肌肉瘤非常罕见，预后差。目的分析原发性下腔静脉(IVC)平滑肌肉瘤患者的手术治疗效果。
方法：回顾性回顾2014年至2023年在一家三级护理医院进行的IVC平滑肌肉瘤切除术。共分析13例,其中包括10名女性和3名男性。出现症状，肿瘤特征，操作管理，术后并发症,评估每位患者的生存率。
结果：患者年龄中位数为59岁（Q1：52，Q3：68）。中位肿瘤大小为7.0cm(Q1:6,Q3:12)。中位有丝分裂率为6/10HPF(Q1:2.5,Q3:15.5)。13例患者肿瘤切除均为阴性,9（69％）具有微观阴性边缘（R0）。无患者淋巴结受累。在4例已经阻塞IVC的肿瘤中，IVC通过结扎进行管理，牛心包补片血管成形术7例，或2例专利IVC的初步修复。7例患者同时行右肾切除术。3例患者行左肾静脉结扎术,但未进行左侧肾切除术.术后并发症包括1例下肢骨筋膜室综合征,两名腿部严重肿胀的患者,还有一个手臂肿胀的病人.30天死亡率为零。使用Kaplan-Meier乘积限制方法，疾病特异性生存率估计为93%.
结论：对于IVC平滑肌肉瘤患者，手术切除是一种可行且有效的肿瘤治疗选择。IVC可以通过结扎安全管理，初级修复,或补片血管成形术取决于IVC的先前开放。
方法：回顾性回顾2014年至2023年在一家三级护理医院进行的IVC平滑肌肉瘤切除术。共分析13例,其中包括10名女性和3名男性。出现症状，肿瘤特征，操作管理，术后并发症,评估每位患者的生存率。
结果：患者年龄中位数为59岁（Q1：52，Q3：68）。中位肿瘤大小为7.0cm(Q1:6,Q3:12)。中位有丝分裂率为6/10HPF(Q1:2.5,Q3:15.5)。13例患者肿瘤切除均为阴性,9（69％）具有微观阴性边缘（R0）。无患者淋巴结受累。在4例已经阻塞IVC的肿瘤中，IVC通过结扎进行管理，牛心包补片血管成形术7例，或2例专利IVC的初步修复。7例患者同时行右肾切除术。3例患者行左肾静脉结扎术,但未进行左侧肾切除术.术后并发症包括1例下肢骨筋膜室综合征,两名腿部严重肿胀的患者,还有一个手臂肿胀的病人.30天死亡率为零。使用Kaplan-Meier乘积限制方法，疾病特异性生存率估计为93%.
结论：对于IVC平滑肌肉瘤患者，手术切除是一种可行且有效的肿瘤治疗选择。IVC可以通过结扎安全管理，初级修复,或补片血管成形术取决于IVC的先前开放。
