Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China; Section of Vascular Surgery, Department of Surgery, Washington University in St. Louis, St. Louis, Mo.
Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
J Vasc Surg Venous Lymphat Disord. 2018 Sep;6(5):636-645. doi: 10.1016/j.jvsv.2018.03.013. Epub 2018 May 18.
We aimed to review our experience in the diagnosis and surgical management of patients diagnosed with inferior vena cava leiomyomatosis (IVL).
We retrospectively evaluated all patients diagnosed with IVL between 1999 and 2015. Patient demographics, diagnostic imaging, operative techniques, and perioperative outcomes were reviewed.
Over the study period, 16 patients with an IVL diagnosis were identified. In all patients, the diagnosis was made with ultrasound and magnetic resonance imaging. In 15 patients who underwent operative intervention, we observed three tumor extension routes from the uterus to the inferior vena cava: (i) via the internal iliac vein, (ii) via the ovarian vein, and (iii) via the anterior sacral vein. Complete tumor removal was achieved in all patients who underwent a one-stage operation (12 patients). Among these patients, antegrade tumor extraction from the right atrium was performed in nine patients, and retrograde extraction from iliac veins was performed in three. A two-stage operation with direct tumor transection and resection was necessary in a subset of patients to facilitate complete resection in one patient, and near-complete resection in two patients. Preoperative imaging and intraoperative findings demonstrated four distinct types of gross tumor morphologies: (i) type A solid cast (43.8%), (ii) type B hallow tube-like (12.5%), (iii) type C thread-like (18.7%), and (iv) type D mixed morphology (25%). Types A and B were the easiest tumor types to extract, and types C and D tumors were more difficult to remove given their fragility. Postoperative surgical pathology confirmed the diagnosis of IVL. All patients recovered successfully with no major complications; there were no deaths. One patient early in our experience had an incomplete resection and developed a recurrence that required re-intervention at 26 months from the initial operation.
IVL can be accurately diagnosed with ultrasound and magnetic resonance imaging. Surgical tumor resection with a one-stage operation can lead to reasonable outcomes and successful cure rates. The surgical plan can be tailored to the type of tumor morphology observed on preoperative imaging.
我们旨在回顾诊断和治疗下腔静脉平滑肌瘤病（IVL）患者的经验。
我们回顾性评估了 1999 年至 2015 年间所有诊断为 IVL 的患者。评估了患者的人口统计学特征、诊断性影像学、手术技术和围手术期结果。
在研究期间，共发现 16 例 IVL 患者。所有患者均通过超声和磁共振成像诊断。在 15 例接受手术干预的患者中，我们观察到肿瘤从子宫向下腔静脉的三种延伸途径：（i）通过髂内静脉，（ii）通过卵巢静脉，和（iii）通过前骶静脉。所有接受一期手术的患者均成功切除了肿瘤（12 例）。在这些患者中，有 9 例从右心房逆行提取肿瘤，3 例从髂静脉逆行提取肿瘤。对于部分患者，需要进行两期手术，直接肿瘤横断和切除，以实现 1 例患者的完全切除和 2 例患者的近乎完全切除。术前影像学和术中发现显示出四种不同的大体肿瘤形态：（i）A型实性铸型（43.8%），（ii）B 型空心管状（12.5%），（iii）C 型线状（18.7%）和（iv）D 型混合形态（25%）。A 型和 B 型肿瘤最容易提取，而 C 型和 D 型肿瘤由于其脆弱性而更难以切除。术后手术病理学证实了 IVL 的诊断。所有患者均成功康复，无重大并发症；无死亡。在我们的早期经验中，有 1 例患者的肿瘤切除不完全，在初始手术 26 个月后复发，需要再次干预。
超声和磁共振成像可准确诊断 IVL。一期手术切除肿瘤可获得合理的结果和较高的治愈率。手术计划可以根据术前影像学观察到的肿瘤形态类型进行定制。
