子宫脉管内平滑肌瘤的相关研究

Related research on uterine intravascular leiomyoma

脉管内平滑肌瘤(IVL)是一种较为罕见的特殊类型的子宫肌瘤,不局限于生长在子宫肌层及子宫静脉内,可以通过髂内静脉等多种途径延伸至心脏甚至肺动脉内,从而出现相应的临床表现,严重威胁患者健康,甚至生命。相较于其他类型子宫肌瘤,IVL临床表现多种多样,误诊率及漏诊率较高,需要多种影像学检查。病理检查可见肿瘤组织周围增生的血管,无非典型细胞及凝固性坏死。免疫组化表现为结蛋白、抗波性蛋白、雌激素、孕酮受体阳性,可用于指导疾病的诊断及辅助治疗。该疾病大多需要手术治疗,但术后的复发率较高,需要适当的药物辅助治疗。术后可辅以促性腺激素释放激素激动剂,大环内脂类免疫抑制剂也有一定的治疗效果。该疾病存在多种染色体及基因改变,其发病基因的研究可进一步提供靶向治疗以预防术后复发。本文对该疾病的最新研究进行综述。

Intravascular leiomyoma(IVL)is a rare and special type of uterine of leiomyoma.The growth of IVL is not limited to the myometrium and uterine veins,and it can extend to the heart and even to the pulmonary arteries through a variety of ways,such as the internal iliac vein and other ways.Thus the corresponding clinical manifestations can seriously threat the health of the patients,or even threat their lives.Compared with those of other types of uterine fibroids,IVL has a variety of clinical manifestations with high rate of misdiagnosis and missed diagnosis,so it requires a lot of imaging examinations for diagnosis.Under the pathological examination microscope,the proliferation of blood vessels around the tumor tissue without atypical cells and coagulative necrosis can be seen.Immunohistochemical performance is positive for desmin,antiwave protein,estrogen,and progesterone receptors,which can be used to guide disease diagnosis and adjuvant treatment.Most of the patients with IVL require surgical treatment,but the postoperative recurrence rate is high,so appropriate drug-assisted treatment for them is required.Gonadotropin-releasing hormone agonists can be assisted after surgery,and macrolide immunosuppressants also have certain therapeutic effect.IVL has diversity of chromosomal and genetic changes,and the study on its genes can further provide targeted therapy and postoperative recurrence prevention.This article mainly reviews the latest research on IVL.