类似卵巢性索肿瘤的子宫肿瘤五例临床病理分析

目的探讨类似卵巢性索肿瘤的子宫肿瘤(UTROSCT)的临床病理特点。方法回顾性分析5例UTROSCT的临床特征,总结其组织学形态及免疫组化表达特点,并进行随访。结果患者年龄40~60岁(平均49.2岁,中位52岁)。3例以阴道出血就诊,1例因子宫肌瘤切除发现,1例因体检发现黏膜下肌瘤就诊。3例肿瘤组织位于内膜部位,1例位于肌壁间,1例位于内膜及肌层。4例患者随访18~147个月不等(平均95.5个月),无一例复发或转移。组织学上可见巢状、小梁状、条索样、片状或Sertorli管样结构;肿瘤细胞可呈上皮样,具有丰富的、泡沫状或透明的胞质,也可呈短梭形或卵圆形,胞质稀少;细胞异型性小,核分裂象少见。免疫组化显示肿瘤细胞表达AE1/AE3(4/4)及vimentin(3/3),不同程度地表达性索标志物α?inhibin(5/5)、calretinin(5/5)、CD99(5/5)、MelanA(2/3),激素受体ER(3/4)、PR(4/4),及CD10(4/5),不表达平滑肌标志物SMA(0/2)。Ki?67增殖指数2%~10%。结论UTROSCT是一类特殊的子宫肿瘤,绝大部分呈良性经过,病理形态具有性索样的分化特点,组织结构多样,可表达多种性索及上皮标记物,分子改变与卵巢性索肿瘤及子宫内膜间质肿瘤不同,可资鉴别。

Uterine tumor resembling ovarian sex cord tumor:a clinicopathologic study of five cases

Objective To investigate the clinicopathologic features of uterine tumor resembling ovarian sex cord tumor(UTROSCT).Methods The clinical,histopathologic and immunohistochemical features of 5 cases of UTROSCT were retrospectively analyzed.Results The patients’ages ranged from 40 to 60 years.Most cases presented with vaginal bleeding(3/5)and most tumors were intramural(3/5).Four follow?up cases were alive and free of disease with a mean follow?up interval of 95.5(range,18-147)months.Histologically,tumors grew in nests,trabeculae,cords,sheets,or Sertoli tubules.Cells had scant to abundant,foamy or clear cytoplasm.Nuclei were ovoid with minimal atypia and low mitotic activity.Tumors were positive for AE1/AE3(4/4),vimentin(3/3),sex cord markers(α?inhibin 5/5,calretinin 5/5,CD995/5,melan A 2/3),ER(3/4),PR(4/4),and CD10(4/5),but negative for actin(0/2).The rate of cell proliferation measured by Ki?67 was 2%-10%.Conclusion UTROSCT is a special type of uterine neoplasm with benign prognosis in most cases.Tumors show heterogeneous sex cord?like features,and have a polyphenotypic immunohistochemical profile,with positivity for various epithelial and sex cord markers.UTROSCT does not harbor the molecular alterations seen in endometrial stromal sarcoma and ovarian sex?cord tumors,which could provide evidences for the differential diagnosis.