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目的探讨类似于卵巢性索肿瘤的子宫肿瘤(UTROSCT)的临床病理特征,避免误诊。方法分析1例UTROSCT的临床资料,进行组织形态学观察、免疫组化标记及基因检测,复习文献。结果本例肿瘤最大径12cm,瘤细胞呈上皮样形态,排列呈小梁状、条索状、巢状、假腺样结构,浸润周围平滑肌组织,可见血管内瘤栓。部分瘤细胞胞质呈泡沫状,部分核仁显著,可见核沟,核分裂象3个/10HPF。免疫组化:CK、CK8、CK19、vimentin、CD99、bcl-2、AR、ER和PR呈弥漫(+),CR、CD56呈灶状(+),inhibin-α、melan-A、WT-1、FOXL2、SF-1、PAX-8、CD10、CK7、CK20、CK5、CK14、CK34βE12、p16、EMA、HMB45、SMA、desmin、calponin、S-100、CD117、CD34、FLI-1、MC、CD133、CD44、Cg A和Syn均(-),p53为野生型,Ki-67增殖指数为5%;FOXL2基因第1外显子及DICER1基因第24-25外显子无突变。子宫切除术后随访4个月,健在。结论 UTROSCT是一种罕见的子宫肿瘤,缺乏FOXL2和DICER1基因突变。诊断需要依靠形态学及免疫组化标记。
Objective To investigate the clinical and pathological features of uterine tumors similar to ovarian tumors (UTROSCT) and to avoid misdiagnosis. Methods The clinical data of 1 case of UTROSCT were analyzed and the histopathological observation, immunohistochemical markers and gene tests were performed and the literature was reviewed. Results The maximum diameter of tumor in this case was 12 cm. The tumor cells showed epithelial morphology with trabecular, cords, nests, and pseudo-gland-like structures infiltrating surrounding smooth muscle tissue. Some tumor cells cytoplasm was foamy, some prominent nucleoli, showing the ditch, mitotic figure 3 / 10HPF. Immunohistochemistry showed diffuse (+), CR, CD56-like, +, inhibin-α, melan-A and WT-1 expressions in CK, CK8, CK19, vimentin, CD99, FOXL2 SF-1 PAX-8 CD10 CK7 CK20 CK5 CK14 CK34βE12 p16 EMA HMB45 SMA desmin calponin S-100 CD117 CD34 FLI-1 MC CD133 , CD44, Cg A and Syn (-), p53 was wild type, Ki-67 proliferation index was 5%; FOXL2 gene exon 1 and DICER1 gene 24-25 exon no mutation. Hysterectomy followed up for 4 months, alive. Conclusion UTROSCT is a rare uterine tumor lacking FOXL2 and DICER1 gene mutations. Diagnosis needs to rely on morphological and immunohistochemical markers.