雄激素不敏感综合征4例临床病理分析

Clinicopathologic analysis of 4 cases of androgen insensitivity syndrome

通过分析4例雄激素不敏感综合征(androgen insensitivity syndrome,AIS)的临床病例特点、鉴别诊断、治疗及预后,综合复习相关文献。4例患者均为完全型AIS,年龄15~33岁,其中2例存在性腺肿瘤,均位于腹腔,肿瘤类型包括精原细胞瘤、Sertoli-Leydig细胞肿瘤(高分化)及纤维瘤(1例),Sertoli细胞腺瘤(1例)。所有患者均行性腺切除手术。AIS的诊断需要结合临床特征、影像学检查及染色体核型分析等方面综合考虑。

The clinicopathologic pathological features,differential diagnosis,treatment and prognosis of 4 analysis cases of androgen insensitivity syndrome(AIS)were investigated and the related literature was reviewed.All 4 patients were complete AIS(CAIS)aged 15 to 33.Two patients were diagnosed with gonadal tumor,all in the abdominal cavity and the tumor types included seminoma,well differentiated Sertoli-Leydig cell tumor and fibrothecoma(1 case)and Sertoli cell tumor(1 case).All patients were performed gonadectomy.The diagnosis of AIS needs combined consideration of clinical features,imaging examination and chromosome karyotype analysis.