血管内平滑肌瘤病21例临床分析

A clinical analysis of 21 intravascular leiomyomatosis cases

目的：探讨血管内平滑肌瘤病（intravascular leiomyomatosis,IVL）的临床病理特征、诊断、治疗方法及预后。方法：回顾性分析中国医科大学附属盛京医院2004年1月至2015年8月收治的21例血管内平滑肌瘤病患者的临床资料,对其临床病理特征、诊断、治疗方法及预后进行分析。结果：21例患者发病年龄27~65岁（平均年龄44.4岁）,中位年龄45岁。女性患者20例,男性患者1例。其中一例为该病术后复发。21例患者无特异性症状及体征,大多为发现盆腔包块入院。IVL术前确诊率极低,主要依靠术中探查及术后病理特征,大体可见血管内条索状、金针菇状或结节状肿物,大小不等,可从血管内抽出,镜下瘤细胞呈梭形,编织状排列,一般无核分裂象。21例患者全部接受手术治疗,术中冰冻15例诊断该病,术后石蜡病理诊断明确。随访18例,中位随访时间为39个月,无1例复发。结论：血管内平滑肌瘤病是一种具有恶性潜能的良性病变,好发于育龄期女性,临床表现无特异性,术前诊断率低,确诊靠术后石蜡病理,以手术治疗为主,以病灶切除干净为原则,因为该病为雌激素依赖性疾病,对于年龄偏大、无生育要求者可以考虑双侧附件同时切除,对于保守治疗的患者,术后可辅助促性腺激素释放激素激动剂（GnRH-a）及其他抗雌激素药物,避免应用雌激素类药物或食物,术后需定期复查,严密随访。本例男性病例为首次报道。

Objective：To investigate the clinicopathological features, diagnosis, treatment and prognosis of intra- vascular leiomyomatosis（IVL）. Methods：Retrospectively analyzed the clinical data of the 21 IVL cases in Shengjing Hospital of China Medical University data 2004 to 2015, the clinical and pathological features, diagnosis, treatment and prognosis were reviewed. Results ：The mean age of the 21 patients was 44.4 years （range：27 years- 65 years）, the median age was 45 years. There were 20 females and 1 male, and one for the recurrence of the disease. Most of the cases showed gradually enlarged pelvic mass and no other obvious symptoms was complained. The clinical diagnosis of IVL patients is difficult before operation, and mainly depends on the intraoperative exploration and pathological exami- nation. The tumors were in clusters or nodules in the vessels, which were white or grey, and can be removed fi＇om the vessels. On the microscopic examination, these tumor cells were spindled, and mitotic activity was typically minima. All these 21 cases received operation,and 15 cases were confirmed by the frozen section examination ,and all of them were confirmed after operation. 18 cases were followed up. Median follow - up time was 39 months, and no one reoccurred. Conclusion：IVL can be histologically benign but biologically malignant, that occurs principally in the reproductive age women, which is confirmed by the pathological diagnosis. The main treatment method is surgical operation, based on the principle of complete removal, and gonadotropin -releasing hormone agonist（ GnRH -a） or other anti - estrogen drug therapy is essential for conservative treatment, a strict follow up is needed.