【摘要】 目的探讨性腺母细胞瘤(GB)的临床病理特征,加强对此疾病的认识。方法采用HE染色、免疫组化SP法对2014—2019年我院确诊的2例GB进行病理学观察,并结合临床资料进行分析。结果患者社会性别为女性,染色体核型均为46(X, Y)。临床表现为原发性闭经、性激素水平异常、性腺发育不全等。肿瘤由巢状分布的原始生殖细胞和周围簇状排列的性索样细胞构成,伴无性细胞瘤或精原细胞瘤成分,可见Call-Exner小体及钙化。免疫组化显示OCT3/4、PLAP、α-inhibin、CD117(+)。结论 GB是一种罕见的性腺肿瘤,常伴性腺发育不全,合并无性细胞瘤或精原细胞瘤。青年女性患者以原发性闭经就诊,应及时进行染色体核型分析和性激素水平检测,并在病理活检中广泛取材以明确诊断,尽早手术切除性腺。更多还原

【Abstract】 Objective To investigate the clinicopathological features of gonadoblastoma and to enhance the understanding of this disease. Methods HE staining and immunohistochemical SP method were used to observe the pathological characteristics in 2 cases of gonadoblastoma diagnosed in our hospital from 2014 to 2019, combined with analyses of clinical data. Results Both patients were female, and the karyotype were 46(X, Y). The clinical manifestations were primary amenorrhea, abnormal sex hormone levels, and hypogonadism. The tumor consisted of nested primordial germ cells and surrounding clusters of sex-cord-like cells, with dysgerminoma or seminoma, showing Call-Exner bodies and calcification. Immunohistochemistry showed OCT3/4, PLAP, α-inhibin, and CD117 were positive. Conclusions Gonadoblastoma is a rare gonadal tumor, and often associated with hypogonadism, combined with dysgerminoma or seminoma. Young women with primary amenorrhea should be diagnosed with karyotype analysis and sex hormone detection in time, and extensive tissue selection in pathological biopsy can be used to confirm the diagnosis and then surgically remove the gonads as soon as possible.更多还原