静脉内平滑肌瘤病累及下腔静脉六例的处理经验

Experience in management of intravenous ieiomyomatosis with inferior vena cava extension

目的评价静脉内平滑肌瘤病累及下腔静脉时的诊断和处理方式。方法回顾性分析了北京协和医院血管外科自2000年7月至2006年2月收治的6例累及下腔静脉的静脉内平滑肌瘤病。结果6例患者中3例行Ⅰ期，2例行Ⅱ期手术，手术均获得成功，无围手术期死亡或并发症，其中4例患者的原发肿瘤和静脉内瘤栓完整切除，1例患者因多次手术，肿瘤与盆腔粘连严重，残余少许肿瘤，但术后给予抗雌性激素治疗后，瘤体有明显缩小。所有患者术后均复发。结论静脉内平滑肌瘤病极为少见，血管外科临床医师应当对该病有足够的重视，该病累及下腔静脉时可以选择多种治疗方式，手术是最佳选择，能否完整切除瘤体是防止复发的关键。

Objective To evaluate the diagnostic and treatment of intravenous leiomyomatosis （IVL） with extension to inferior vena cava. Methods The clinical data of 6 cases of IVL involving inferior vena cava were analyzed retrospectively. Results Three of the 6 patients underwent phase Ⅰ operation, and 2 underwent phase Ⅱoperation, all successfully; and 1 did not undergo operation sue to poor cardiopulmonary function. No peri-operative death and complication were observed. The primary tumor and intravenous tumor embolus were completely resected in 4 out of the 6 patients. One patient who had serious adherence between the tumor and pelvic cavity due to multiple operations had some residual tumors left. However, after anti-estrin treatment, the residual tumors had obviously regressed. All the patients had relapsed after the operation. Conclusion IVL is very rare. The vascular surgeon must pay more attention to this disease. There are many therapeutic methods to choose when the IVL involves the inferior vena cava, among which operation is the best choice. Completely resection of the tumor is the key of the prevention of recurrence.