2018 年第 10 期 第 13 卷

静脉内平滑肌瘤病累及下腔静脉或右心系统的临床特征分析

Clinical features of intravenous leiomyomatosis with inferior vena cava extension or intracardiac extension

作者：张蔚聪孔璐芦艳科雨彤武迎吴山李晓明赵青汪振佳张纯

英文作者：

单位：100029首都医科大学附属北京安贞医院超声科（张蔚聪、科雨桐、吴山、李晓明、赵青、张纯），病理科（武迎），影像科（汪振佳）；100069北京，首都医科大学基础医学院生化教研室（孔璐）；072150河北省保定第七医院超声科（芦艳）

英文单位：

关键词：静脉内平滑肌瘤；下腔静脉；右心系统

英文关键词：

• 摘要：

• 【摘要】目的    探讨静脉内平滑肌瘤病(IVL)累及下腔静脉（IVCE）或IVL累及右心系统（ICE）的临床诊断指标、治疗方法以及预后情况。方法    选取2006年1月30日至2018年1月30日首都医科大学附属北京安贞医院收入的IVCE和ICE患者21例，其中IVCE患者7例，ICE患者14例，对患者超声检查结果、CT检查结果、病理结果、手术及随访结果进行回顾性分析。结果    21例患者均接受超声检查，所有肿物均起源于下腔静脉，其中14例可见肿物侵入右心系统，7例位于右心房内，6例从右心房侵入右心室，1例侵入到肺动脉造成肺栓塞。21例患者中16例实施了CT检查，14例患者CT显示蛇形的下腔静脉内充盈缺损延伸入右心系统，充盈缺损位于管腔的中央。共16例患者接受病理检查，其中13例可见肿瘤细胞包绕侵袭血管，5例间质血管丰富，可见较多薄壁血管及厚壁血管。入院的21例患者中18例进行手术治疗，3例患者未进行手术。3例未手术患者中1例失访，1例猝死，1例症状无缓解。18例接受手术患者中，2例仅接受分期一期手术治疗的患者出现复发，其中1例患者因继发肠梗阻而死亡，余16例患者均未见复发迹象。结论    超声检查对ICVE或ICE的早期诊断起关键作用，CT对术前整体评估及全面诊断有很大的帮助，外科手术是最主要的治疗方式，完全彻底地切除肿物对改善患者预后至关重要。

• 【Abstract】Objective    To explore the diagnosis, therapy and prognosis of intravenous leiomyomatosis(IVL) with inferior vena cava extension（IVCE） or intracardiac extension（ICE）. Methods    Clinical data of 21 IVL patients(7 IVCE and 14 ICE) admitted to Beijing Anzhen Hospital, Capital Medical University from January 30th 2006 to January 30th 2018 were retrospectively analyzed. Ultrasonic and CT manifestations, pathological results, surgical and follow-up records were analyzed. Results    Ultrasonography showed that IVL derived from inferior venae cava in 21 patients; 14 patients had ICE, including 7 cases in right atrium, 6 cases in right atrium and ventricle, 1 case in pulmonary artery. CT examination was performed in 16 patients; 14 cases showed filling defect in inferior venae cava and right heart. Pathological examination was performed in 16 patients; 13 cases showed tumor cells infiltrating vascular lesions; 5 cases showed large amount of interstitial vessels. In 3 patients without surgery, 1 patient was lost to follow-up; 1 patient suffered from sudden death; 1 patient showed no improvement during follow-up. In 18 patients treated with surgery, 2 patients with stage-one operation had relapse; 1 patient died of intestinal obstruction; 16 patients had no relapse during follow-up. Conclusions    Echocardiography is an essential mean for the early diagnosis of ICVE and ICE. CT may help preoperative evaluation and comprehensive diagnosis. Surgical treatment should be performed to thoroughly remove the tumor, which is critical to prevent recurrence and improve the prognosis of patients.