类似于卵巢性索肿瘤的子宫肿瘤临床病理观察  

Clinicopathologic features of uterine tumor resembling ovarian sex-cord tumor

作　　者：刘晓刚 陶祥[2] 刘岩[3] 张新鹏 薛慧忠 孔令红 蔡辉 LIU Xiao-gang TAO Xiang LIU Yan ZHANG Xin-peng XUE Hui-zhong KONG Ling-hong CAI Hui(Department of Pathology, Beijing Chuiyangliu Hospital, Beijing 100022, China Department of Pathology, Obstetrics and Gynecology Hospital of Fudan University, Shanghai 200011, China Department of Pathology, Peking University Health Science, Beijing 100191, China)

机构地区：[1]垂杨柳医院病理科,北京100022 [2]复旦大学附属妇产科医院病理科,上海200011 [3]北京大学医学部病理学系,北京100191

出　　处：《诊断病理学杂志》2017年第7期527-531,共5页Chinese Journal of Diagnostic Pathology

基　　金：北京市垂杨柳医院硕博基金(编号2015-02)

摘　　要：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 clinicopathologic features of uterine tumor resembling ovarian sex-cord tumor（ UTROST） to avoid misdiagnosis. Methods The clinical findings,morphologic features,immunohistochemical markers and gene mutation analysis in one case of UTROSCT were studied. Results A 20-year-old student presented with abnormal vaginal bleeding for one year and anemia for three months. The tumor was about 12 cm in maximum diameter with yellow appearance. Microscopically,the tumor had cord,trabeculae,nest,solid,and poseudo-adenoid pattern; the tumor cells were epithelioid partly with foam-like cytoplasm, prominent nucleoli and mitotic figures（ 3/10 HPF）. The immunohistochemical staining showed that tumor cells were positive for CK,CK8,CK19,vimentin,CD99,bcl-2,AR,ER and PR,focal positive for calretinin and CD56,but negative for Inhibin-α,Melan-A,WT-1,FOXL2,SF-1,S-100,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 showed wild type and Ki-67 index number was about 5 percent.FOXL2 and DICER1 mutation were absent. The patient was alive after follow up for 4 months after hysterectomy.Conclusions UTROSCT is a very rare tumor and FOXL2 and DICER1 mutation might be absent. The definite diagnosis depends on morphological observation and immunohistochemical analysis.

关 键 词：类似于卵巢性索肿瘤的子宫肿瘤 免疫学表型 基因突变 

分 类 号：R737.33[医药卫生—肿瘤]