不同类型雄激素不敏感综合征性腺肿瘤临床分析  

The Gonadal Tum or Risk in Different Types of Androgen Insensitivity Syndrome

作　　者：蒋建发[1] 薛薇[1] 邓燕[1] 王艳芳[1] 田秦杰[1] 孙爱军[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院,北京100730

出　　处：《实用妇产科杂志》2016年第2期110-112,共3页Journal of Practical Obstetrics and Gynecology

摘　　要：目的：探讨完全型雄激素不敏感综合征（CAIS）与不完全型雄激素不敏感综合征（IAIS）患者的性腺肿瘤发生情况及临床特征,以进一步提高对该病的认知水平与诊治。方法：回顾性分析2001年1月至2015年3月北京协和医院妇产科收治的雄激素不敏感综合征（AIS）78例患者的临床资料及性腺病理检查结果。结果：78例AIS患者中,CAIS 52例,IAIS 26例。共14例（17.9%,14/78）患者存在性腺肿瘤,年龄14~33岁,肿瘤类型包括：性腺母细胞瘤（2例）、支持细胞腺瘤（8例）、精原细胞瘤（1例）、无性细胞瘤（1例）和性索肿瘤（1例）、左侧性腺精原细胞瘤及右侧支持细胞腺瘤（1例）。发生肿瘤的性腺位于腹盆腔9例,腹股沟管5例。14例性腺肿瘤患者诊断为CAIS12例和IAIS 2例,其性腺肿瘤的发生率分别为23.1%和7.7%,差异有统计学意义（P〈0.05）。结论：AIS患者性腺易发生肿瘤,非阴囊内部位的性腺易发生性腺肿瘤。CAIS的性腺肿瘤发生率较高。Objective： To ananlyze the gonadal tumor risk in complete androgen insensitivity syndrome（ CAIS）and incomplete androgen insensitivity syndrome（ IAIS） and to increase the knowledge of these diseases. M ethods： We retrospectively analyzed the clinical data and results of gonadal histopathology of patients with androgen insensitivity syndrome adimitted to Peking Union Medical College Hospital from January 2001 to March 2015. Results： We included 52 cases with CAIS and 26 cases with IAIS in the current study. Fourteen patients were diagnosed with gonadal tumor,and the tumor types included gonadoblastoma（ 2 cases）,sertoli cell tumor（ 8 cases）,seminoma（ 1 case）,dysgerminoma（ 1 case） and sex cord tumor（ 1 case）. The gonads of 9 patients with gonadal tumor located in abdominal and pelvic,the rest 5 cases located in inguinal canal. The total tumor risk was 17. 9%and the gonadal malignancy risk in patients with CAIS and IAIS was 23. 1% and 7. 7%,respectively. The difference in the tumor risk between the groups was significant（ P〈0. 05）. Conclusions： The gonads of patients with AIS and the gonads not in scrotum have high risks of gonadal tumor. The tumor risk in women with CAIS is high.

关 键 词：完全型雄激素不敏感综合征 不完全型雄激素不敏感综合征 性腺 肿瘤 

分 类 号：R711[医药卫生—妇产科学]