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目的探讨子宫静脉内平滑肌瘤病(IVL)的临床生物学行为、病理学特点及其免疫表型的临床意义。方法应用免疫组化方法检测子宫IVL肿瘤细胞vimentin、desmin、SMA、CD10、CD34、ER和PR的表达情况。结果6例患者术前均诊断为子宫多发性平滑肌瘤,未曾怀疑为IVL;3例行全子宫切除术,3例行全子宫及双侧附件切除术,其中1例术中见灰白色条索样肿物蔓延致右侧宫角和宫旁组织静脉内;病理检查发现6例标本除合并血管外子宫平滑肌瘤外,子宫肌壁间大小不一的静脉内见平滑肌细胞构成的瘤栓。6例患者经随访6～51个月未见复发。免疫组化染色显示,IVL瘤细胞vimentin、desmin和SMA(+),CD10和CD34(-);其中5例肿瘤细胞ER和PR呈强(+),1例呈(+)。结论IVL是组织学表现为良性却具有类似恶性肿瘤生长方式的一类罕见肿瘤,有远期复发的倾向。IVL肿瘤细胞存在雌、孕激素高表达特性,提示卵巢去势治疗或术前、术后应用雌激素拮抗剂可能是有效的辅助治疗手段。
Objective To investigate the clinical biological behavior, pathological features and immunophenotype of uterine venous leiomyomatosis (IVL). Methods The expression of vimentin, desmin, SMA, CD10, CD34, ER and PR in IVL tumor cells were detected by immunohistochemistry. Results All 6 patients were diagnosed as multiple myoma of the uterus without IVL. Three cases underwent hysterectomy and three cases underwent hysterectomy and bilateral accessory surgery. Among them, one case showed gray-white cords Like the tumor spread to the right side of the uterine horn and uterine tissue within the vein; pathological examination found that 6 cases of specimens combined with extravascular uterine leiomyoma, the uterine muscle wall between the different sizes of intravenous smooth muscle cells seen in tumor emboli. Six patients were followed up for 6 to 51 months without recurrence. The expression of vimentin, desmin, SMA (+), CD10 and CD34 (-) in IVL tumor cells were detected by immunohistochemistry. The ER and PR of 5 tumor cells were strongly (+) and 1 (+). Conclusions IVL is a rare type of tumor histologically characterized as having benign but similar malignant growth and has a tendency of recurrence. IVL tumor cells have high expression of estrogen and progesterone, suggesting that ovariectomized or preoperative and postoperative estrogen antagonists may be effective adjuvant therapy.