Yang Bin, Zhao Xun, Wang Guoliang, Zhang Hongxian, Ma Lulin, Liu Lei, Zhang Shudong
Department of Urology, Peking University Third Hospital, Beijing, 100191, PR China.
BMC Cancer. 2025 Jul 29;25(1):1241. doi: 10.1186/s12885-025-14287-6.
Retroperitoneal vascular leiomyosarcoma (RVLMS), which originates from vascular wall smooth muscle cells, typically requires inferior vena cava (IVC) reconstruction during radical surgery.
A retrospective cohort study was conducted on 24 patients who underwent primary resection of RVLMS from June 2015 to November 2023 in one institution. The patient details, operative management, and follow-up data were assessed.
Regarding the imaging classification of RVLMS, 6 patients were intraluminal type, 9 patients were intermediate type, 4 patients were extraluminal type, and 5 patients were peripheral type. The median tumor size was 80 mm (interquartile range, IQR 63-105 mm). The median operative time was 294 min. The median blood loss was 650 ml. There were significant differences in operation time, blood loss, blood transfusion, and Intensive Care Unit admission rate among the four types of RVLMS. The procedures of vascular reconstruction included primary repair (n = 15), patch angioplasty (n = 2), and IVC ligation (n = 4). 3 patients suffered an R1/R2 margin. With a median follow-up time of 12.5 months, 5 patients developed local recurrence while 7 patients developed distant metastasis. 1 patient had both local recurrence and distant metastasis. The median disease-free survival was 19.0 months (IQR 7.0-59.0 months).
A reasonable surgical strategy of vascular resection and reconstruction in the context of RVLMS surgery was of value in achieving good postoperative outcomes and long-term survival. The imaging classification of RVLMS might help to evaluate the surgical complexity and the prognosis.
腹膜后血管平滑肌肉瘤（RVLMS）起源于血管壁平滑肌细胞，根治性手术通常需要进行下腔静脉（IVC）重建。
对2015年6月至2023年11月在一家机构接受RVLMS初次切除的24例患者进行回顾性队列研究。评估患者详细信息、手术管理及随访数据。
关于RVLMS的影像学分类，腔内型6例，中间型9例，腔外型4例，外周型5例。肿瘤大小中位数为80mm（四分位间距，IQR 63 - 105mm）。手术时间中位数为294分钟。失血中位数为650ml。四种类型的RVLMS在手术时间、失血量、输血情况及重症监护病房入住率方面存在显著差异。血管重建手术包括一期修复（n = 15）、补片血管成形术（n = 2）及IVC结扎（n = 4）。3例患者切缘为R1/R2。中位随访时间为12.5个月，5例患者出现局部复发，7例患者出现远处转移。1例患者同时出现局部复发和远处转移。无病生存期中位数为19.0个月（IQR 7.0 - 59.0个月）。
RVLMS手术中合理的血管切除和重建手术策略对于实现良好的术后效果和长期生存具有重要价值。RVLMS的影像学分类可能有助于评估手术复杂性和预后。
