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作 者:孔令红[1] 刘晓刚[1] 焦阳[1] 曹智 张新鹏[1] 杨晓琳 KONG Ling-hong;LIU Xiao-gang;JIAO Yang;CAO Zhi;ZHANG Xin-peng;YANG Xiao-lin(Department of Pathology,Beijing Chuiyangliu Hospital,Beijing 100022,China;Department of Pathology,Beijing Chaoyang District Maternal and Child Health Care Hospital,Beijing 100021,China;Department of Radiology,Beijing Chuiyangliu Hospital,Beijing 100022,China)
机构地区:[1]北京市垂杨柳医院病理科,北京100022 [2]北京市朝阳区妇儿医院病理科,北京100021 [3]北京市垂杨柳医院放射科,北京100022
出 处:《诊断病理学杂志》2022年第1期1-5,共5页Chinese Journal of Diagnostic Pathology
基 金:北京市朝阳区科技计划项目(CYSF171303)。
摘 要:目的探讨卵巢Sertoli⁃Leydig细胞瘤(SLCTs)的临床表现、病理学特征,并研究其诊断和鉴别诊断要点及临床意义。方法回顾性研究2006⁃02—2020⁃0613例卵巢SLCTs病例,分析其临床特点,进行病理形态观察、免疫组化标记及基因突变检测,并复习相关文献。结果13例患者年龄28~83岁,平均年龄(50.6±16.1)岁,12例阴道异常出血、1例闭经,3例血清AFP升高,均发生于单侧卵巢,肿瘤最大径2~17 cm,平均最大径(8.61±4.66)cm。肿瘤切面实性或囊实性,质中,鱼肉样,色淡黄、灰粉,偶见出血及坏死。镜下肿瘤组织由不同比例的Sertoli细胞和Leydig细胞组成,中分化者10例,低分化者3例,均未见异源性成分及网状结构。免疫组化显示CK、Vimentin、CD99、SF⁃1、Calretinin、α⁃Inhibin、WT⁃1、CD56、PR、MSH2、MSH6、MLH1、PMS2、MelanA、AR呈不同程度阳性,CgA、Syn、AFP、ER、SMA阴性,Ki⁃67增殖指数5~40%。网状纤维染色显示Sertoli细胞巢周围阳性。1例标本检测出DICER1基因点突变(c.5113 G>A))。11例患者实施全子宫+双附件切除术,FIGO分期均为Ⅰa期,2例患者实施单侧附件切除术,10例术后实施辅助化疗,随访患者2~78个月,均无瘤存活。结论SLCTs是较罕见的卵巢性索⁃间质来源的肿瘤,预后大都良好,免疫组化染色在诊断及鉴别诊断中具有重要意义。Objective To explore the clinical manifestations,pathological features of Sertoli⁃Leydig cell tumors(SLCTs),and to research the key points of diagnosis and differential diagnosis and the prognosis.Methods 13 cases of SLCTs from February 2006 to June 2020 were prospectively analyzed on clinical manifestation,stage,pathological features,treatment and prognosis.Result The age of patients ranged from 28 to 83 years,(50.6±16.1)years on average.All tumors are unilateral,12 cases of patients presented with abnormal vaginal bleeding and one case presented with amenorrhoea.3 cases showed AFP elevated in serum.The size of tumors ranged from 2 to 17 cm,(8.61±4.66)cm on average.They were solid or solid and cystic,yellow and pink,occasionally with haemorrhage or necrosis.Tumors composed of variable of sertoli cells,leydig cells and primitive gonadal stroma without retiform and heterologous elements,10 cases of SLCTs were moderate differentiation and 3 cases of SLCTs were poorly differentiation according to WHO standards.Immunohistochemical study showed the tumor cells were positive for CK,Vimentin,CD99,SF⁃1,Calretinin,α⁃Inhibin,WT⁃1,CD56,PR,MSH2,MSH6,MLH1,PMS2,MelanA and AR,while negative for EMA,CgA,Syn,ER,AFP and SMA,proliferation index was from 5%to 40%.Reticular fiber staining showed that it was positive around the sertoli cells nest.One case showed DICER1 gene point mutation(c.5113 G>A),FOXL2 gene mutation was absent.Follow⁃up 2⁃78 months,patients survived without tumor after surgery and adjuvant chemotherapy.Conclusion Ovarian sertoli⁃Leydig cell tumors were rare sex cord⁃stromal tumors with good prognosis for most patients.Immunohistochemical study were helpful to make correct diagnosis and treatment.
关 键 词:卵巢肿瘤 Sertoli⁃Leydig细胞瘤 病理 治疗及预后
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