西藏地区雄激素不敏感综合征伴双侧盆腔隐睾精原细胞瘤一例  

Androgen Insensitivity Syndrome with Bilateral Cryptorchidism and Seminoma in Tibet:Report of One Case

作　　者：魏倩[1] 达珍[1] 次仁曲珍 霍真[2] 左鹏 WEI Qian;DA Zhen;CIREN Quzhen;HUO Zhen;ZUO Peng(Department of Pathology,Tibet Autonomous Region People’s Hospital,Lhasa 850000,China;Department of Pathology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China;Department of Obstetrics and Gynaecology,Peking University People’s Hospital,Beijing 100044,China)

机构地区：[1]西藏自治区人民医院病理科,拉萨850000 [2]中国医学科学院,北京协和医学院,北京协和医院病理科,北京100730 [3]北京大学人民医院妇产科,北京100044

出　　处：《中国医学科学院学报》2022年第1期173-176,共4页Acta Academiae Medicinae Sinicae

摘　　要：雄激素不敏感综合征(AIS)伴双侧盆腔内睾丸恶变的病例非常罕见,需要结合临床表现、体格检查、血清学表现、染色体核型分析及病理表现才能确定诊断。现报告1例发生于西藏地区藏族人群的完全型AIS病例,该病例从发现子宫先天性缺如到双侧盆腔肿物切除历时17年,病理证实双侧盆腔内占位为发育不良的睾丸组织伴精原细胞瘤和支持细胞腺瘤样结节形成。本文总结此类病变的临床病理特征,旨在加深对该疾病的认识。Androgen insensitivity syndrome(AIS)with bilateral testicular malignant transformation is very rare,and its diagnosis should be based on clinical manifestations,physical examination,serological findings,karyotype analysis,and pathological findings.This study reported a case of complete androgen insensitivity syndrome among Tibetan in Tibet.It took 17 years from the discovery of congenital absence of uterus to bilateral pelvic mass resection.Pathological examination confirmed that bilateral pelvic space occupying lesions were dysplastic testicular tissue with seminoma and sertoli cell adenoma-like nodules.This study summarized the clinicopathological features to deepen the understanding of the disease.

关 键 词：西藏 雄激素不敏感综合征 盆腔 隐睾 精原细胞瘤 

分 类 号：R736.8[医药卫生—肿瘤]