伴有静脉瘤栓的子宫静脉内平滑肌瘤病的MSCT及MPR重建表现与鉴别

Intravenous Leiomyomatosis with Inferior Vena Cava and Right Atrium Extension

目的:探讨子宫静脉内平滑肌瘤病(IVL)的临床和影像特点,增进对该病的认识。方法:回顾性分析我院近5年3例伴有下腔静脉及右心受累的子宫静脉内平滑肌瘤病,对其临床特点、影像表现、治疗及预后进行总结。结果:3例患者中2例既往有子宫肌瘤切除史,1例首次发病。2例因下肢水肿、夜间呼吸困难,1例因左协肋部不适发现肾盂积水就诊。CT扫描均发现腹盆腔巨大肿块,表面分叶状,增强多发结节样强化,同时见盆腔静脉增多增粗,静脉内瘤栓形成并经髂内静脉、髂总静脉蔓延至下腔静脉,1例达右心房并引起肝淤血表现。结论:IVL是少见变异的子宫肌瘤,病变可由盆腔静脉向上蔓延至下腔静脉甚至右心房,对生命造成一定威胁,应提高对IVL的认识。

objective： Exploring the clinical, radiologic features of uterine intravenous leiomyomatosis （IVL） to promote awareness of the disease. Methods： 3 cases of IVL with inferior vena cava and right atrium involvement were retrospectively analyzed. Results： Among the 3 patients, 2 had previous hysteromyomectomy history, one is first onset. Two patients went to hospital because of lower extremity edema and nocturnal dyspnea, and one due to hydronephrosis. CT scan found huge abdominal and pelvic masses with lobulated surfaces and multiple nodular enhancements, meanwhile increased and widen pelvic veins were visualized with tumor thrombus formation and extending from the internal iliac vein into common iliac vein and then the inferior vena cava. In one case the tumor thrombus even spreads into the right atrium and causes liver congestion. Conclusion： IVL is a rare variant of uterine fibroids with different characteristics of incidence, imaging findings and course. The tumor may spread from pelvic veins upward to the inferior vena cava or even right atrium causing threat to patient＇s life. It is important to raise the awareness of IVL.