子宫静脉内平滑肌瘤病临床病理与生物学行为分析

Intravenous leiomyomatosis of uterus: Clinicopathologic analyses and biologic behaviors evaluation

目的观察和分析子宫静脉内平滑肌瘤病(intravenous leiomyomatosis of the uterus,IVL)的临床和病理学特征,期望更好地了解其生物学行为,为临床和病理诊断提供帮助。方法回顾分析29例IVL的临床和病理资料,采用LABC法免疫组化检测desmin、SMA、CD10、vimentin、CD34、ER、PR表达。结果所有患者术前均诊断为子宫平滑肌瘤或子宫腺肌病,无1例怀疑为静脉内平滑肌瘤病。手术中,29例均行冷冻切片检查且均正确诊断为子宫静脉内平滑肌瘤病;所有病例术后经石蜡包埋病理切片检查确诊。28例全子宫切除标本中,肿块均沿脉管腔穿出宫旁或浆膜下。获访的18例患者中,1例患者在肌瘤剥出术后两次复发,行全子宫+双侧附件切除术后随访至今无复发,其余患者随访无复发。免疫组化染色显示脉管内的肿瘤细胞desmin、SMA均阳性;脉管内皮细胞CD34阳性。结论正确认识这种少见肿瘤的大体和剖面特点可减少误诊率,术中冷冻切片检查有助于提高术中诊断率和正确选择处理方式;由于肿瘤有潜在的复发性,患者术后长期随访十分必要。

Purpose To investigate the biologic behavior of intravenous leiomyomatosis （IVL） of the uterus and to gather a better understanding of its patterns of diagnosis. Methods 29 cases of IVL were reviewed and analyzed. Results During the operation, a frozen section was requested for examination, revealing intravenous leiomyomatosis in 29 cases. All the cases were diagnosed as IVL correctly and further confirmed by pathologic analysis of paraffin-embedded histologic sections. In the hysterectomy specimen,28 cases of tumor extended along venous lumina to parametrium or extrauterine with grape-like nodules. Histologic examination revealed a corresponding range of morphologic variants as reported in the leiomyomas. Immunohistochemically,the intravascular parts of the tumor revealed a positive staining reaction for desmin and SMA, The endothelium of vessels reacted with antibody against CD34. Of the 17 patients with follow-up were now disease free, and in one patient, residual tumor was detected at hysterectomy and left-lateral salpingo-oophorectomy 2 years after myomectomy, and pelvic mass was found 2 years after the second operation, right-lateral salpingo-oophorectomy was performed and pelvic mass was resected. This patient was alive and disease free 5 years postoperatively. Conclusions Knowledge about this rare uterine tumor is important for adequate treatment and exact diagnosis, and pathologic examination of frozen section may help improve the rate of correct diagnosis of IVL. Since intravenous leiomyomatosis demonstrates a tendency to recur, long-term follow-up of the patient is recommended.