目的探讨性腺母细胞瘤的临床和病理组织学特征及可能的病因。方法对9例性腺母细胞瘤的临床、病理学特征以及免疫组化结果进行分析。结果患者均为女性,以原发性闭经、两性畸形为主要临床表现,但染色体检查发现均有Y染色体,卵泡刺激素(FSH)和黄体生成素(LH)显著升高,睾酮(T)和雌二醇(E2)水平下降,提示性腺功能不良。肿瘤中可见巢状分布的原始生殖细胞、颗粒样细胞及Call-Exner样小体,其中2例合并无性细胞瘤成分,1例合并原位精原细胞瘤。免疫组化:PLAP和α-inhibin(+)。结论性腺母细胞瘤是一种罕见的良性肿瘤,好发于性发育异常者,可合并无性细胞瘤及精原细胞瘤,治疗采取手术切除性腺,术后辅助性激素治疗。 Objective To investigate the clinical and histopathological features and possible causes of gonadoblastoma. Methods Nine cases of gonadoblastoma clinical and pathological features and immunohistochemical results were analyzed. Results All of the patients were female. Primary amenorrhea and hermaphroditism were the main clinical manifestations. However, chromosome Y was found to have Y chromosome, follicle stimulating hormone (FSH) and luteinizing hormone (LH) were significantly increased, while testosterone Estradiol (E2) levels decline, suggesting that gonadal dysfunction. Neoplasms, granulocyte-like cells and Call-Exner-like bodies were found in neoplasms. Two of them were associated with dysgerminoma and one had primary seminoma. Immunohistochemistry: PLAP and α-inhibin (+). Conclusion Glioblastoma is a rare benign tumor, which occurs in patients with abnormal sexual development. It can be combined with dysgerminoma and seminoma. Surgical gonadal resection and postoperative adjuvant hormone therapy are used.