子宫静脉内平滑肌瘤病病理特征

Clinicopathological characteristics of uterine intravenous leiomyomatosis

目的分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis,IVL)的临床病理特征。方法分析33例IVL患者临床表现,对其手术标本进行常规H-E染色及免疫组织化学En Vision法染色观察。结果患者平均年龄47岁,以盆腔肿块及月经异常为主要临床表现。术前影像学及超声检查均未提示IVL。术后标本检查肿块主体直径平均6 cm,多为水肿,半透明,粘冻状,质软,略不规则,周围肌层内见肿块呈蠕虫样或串珠样组织穿梭,有19例自宫体向宫旁及阔韧带穿出。光镜下肿瘤由典型的梭形平滑肌细胞组成,细胞形态温和,异型性不明显,无坏死,核分裂象罕见,伴透明样变及黏液样变,肿瘤表面覆盖一层扁平的血管内皮细胞。随访时间为6~108个月,有1例转移至肺部,3例反复复发。免疫组化染色显示肿瘤细胞ER、PR、SMA、Caldesmon、Desmin及脉管内皮细胞CD34/CD31均阳性。结论子宫静脉内平滑肌瘤病是一种少见的子宫平滑肌肿瘤,熟悉其特征性表现,仔细检查并全面取材可减少漏诊率,正确认识这种肿瘤的特点对病理诊断及临床选择合适的手术方式极为重要。

Objective To analyze the clinicopathological features of uterine intravenous leiomyomatosis（ IVL）. Methods The clinical data of 33 patients with IVL were analyzed. The specimens were examined by routine H-E staining and immunohistochemical En Vision staining. Results The median age of patients was 47 years old. The main clinical manifestations were pelvic masses and abnormal menstruation.Preoperative imaging examinations were not suggestive of IVL in all cases. Postoperative specimen examinations showed that the average diameter of the myoma was 6 cm,the tumors were translucent,sticky,soft and slightly irregular with edema. In the surrounding muscular layer,there were worm-like or beaded-like tissues. The tumors traversed to the outside of uterus and the broad ligament in 19 cases.The light microscopy showed that the tumors were composed of typical spindle-shaped smooth muscle cells,the cells morphology was mild,atypias were not obvious and mitoses were rare; there was no necrosis,but there were hyaline and myxoid changes. The tumor surfaces were covered with a layer of flat endothelial cells. Patients were followed up for 6 ~ 108 months,metastasis to the lungs was observed in 1 case,while repeat recurrences were observed in 3 cases. Immunohistochemical staining revealed positive ER,PR,SMA,caldesmon,desmin and vascular endothelial cell CD34/CD31 in tumor cells. Conclusion Uterine IVL is a rare variant of leiomyoma. Understanding the features of IVL is important for patholo gical diagnosis and appropriate clinical treatment.