Wen Yulin, Ma Guotao, Miao Qi, Shao Jiang, Lu Wei, Liu Xingrong, Zhang Chaoji, Liu Jianzhou, Cao Dongyan, Chen Ninghai, Wang Jinhui
Department of Cardiac Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Cardiac Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
J Vasc Surg Venous Lymphat Disord. 2025 Jan;13(1):101989. doi: 10.1016/j.jvsv.2024.101989. Epub 2024 Oct 11.
Intravenous leiomyomatosis (IVL) is a rare neoplasm; the accumulated knowledge about the characteristics and prognosis of this tumor has been derived mainly from isolated case reports with no comprehensive research. In this study, we reviewed our institution's experience with IVL over a 20-year period and developed a classification system that can be used to guide surgical management.
The study had a retrospective cohort design and included patients who underwent resection of IVL at our institution between January 2002 and December 2022. Perioperative parameters were then collected among four stages of our proposed classification. The long-term outcomes, oncologic prognosis, and factors associated with recurrence were analyzed.
A total of 216 patients were included (stage 1, n = 92; stage 2, n = 39; stage 3, n = 76; stage 4, n = 9). The mean follow-up duration was 26.34 months, during which 18 patients (9.7%) in the complete resection group had recurrence, and 12 (39.0%) in the incomplete resection group showed disease progression. Recurrence or progression of residual disease was associated with adjuvant aromatase inhibitor therapy and maximum tumor thrombus diameter but not with total hysterectomy and bilateral salpingo-oophorectomy, age, or postoperative treatment with a gonadotropin-releasing hormone agonist therapy.
This is the largest single-center report on IVL published to date and provides valuable information on its clinical features and long-term outcomes, as well as surgical technique. Our classification system can be used to evaluate the extent of lesion involvement and guide surgical management.
静脉内平滑肌瘤病（IVL）是一种罕见的肿瘤；关于该肿瘤特征和预后的累积知识主要来自孤立的病例报告，缺乏全面研究。在本研究中，我们回顾了本机构20年来IVL的治疗经验，并开发了一种可用于指导手术管理的分类系统。
本研究采用回顾性队列设计，纳入2002年1月至2022年12月在本机构接受IVL切除术的患者。然后在我们提出的分类的四个阶段收集围手术期参数。分析长期结局、肿瘤预后以及与复发相关的因素。
共纳入216例患者（1期，n = 92；2期，n = 39；3期，n = 76；4期，n = 9）。平均随访时间为26.34个月，其中完全切除组18例患者（9.7%）复发，不完全切除组12例患者（39.0%）疾病进展。残留疾病的复发或进展与辅助芳香化酶抑制剂治疗和最大肿瘤血栓直径有关，但与全子宫切除术和双侧输卵管卵巢切除术、年龄或促性腺激素释放激素激动剂治疗的术后处理无关。
这是迄今为止发表的关于IVL的最大规模单中心报告，提供了关于其临床特征、长期结局以及手术技术的宝贵信息。我们的分类系统可用于评估病变累及范围并指导手术管理。
