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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 intravascular leiomyomatosis (IVL). Methods: The clinical data of 21 patients with intravascular angiomyolipoma who were admitted to Shengjing Hospital of China Medical University from January 2004 to August 2015 were retrospectively analyzed. The clinicopathological features, diagnosis, treatment and prognosis were analyzed . Results: The age of onset of 21 patients was 27-65 years (mean age 44.4 years), the median age was 45 years. 20 female patients and 1 male patient. One case of the disease recurrence. 21 patients with no specific symptoms and signs, mostly found pelvic mass admission. IVL preoperative diagnosis rate is very low, mainly by intraoperative exploration and postoperative pathological features, generally visible intravascular cords, pinnules or nodular masses, ranging in size, can be drawn from the blood vessels, microscopic tumor cells Fusiform, woven arrangement, the general non-mitotic figure. Twenty-one patients underwent surgical treatment. Intraoperative frozen in 15 cases diagnosed the disease, postoperative pathological diagnosis of paraffin clear. Follow-up 18 cases, the median follow-up time was 39 months, no recurrence in 1 case. Conclusion: Endovascular leiomyoma is a benign disease with malignant potential, which occurs in women of childbearing age. The clinical manifestations are nonspecific, the preoperative diagnosis rate is low, and the pathological diagnosis of postoperative paraffin pathology is based on surgical treatment. To clean the principle of removal of the lesion, because the disease is estrogen-dependent disease, for older, no requirements of fertility may consider simultaneous removal of bilateral attachments, for patients with conservative treatment, postoperative adjuvant gonadotropin-releasing hormone agonist (GnRH-a) and other anti-estrogen drugs to avoid the application of estrogen drugs or food, regular review after surgery, close follow-up. This case is the first case of male cases.