46XY simple gonadal hypoplasia: 2 case reports and literature review
10.3760/cma.j.cn101721-20230709-000241
- VernacularTitle:46XY单纯性腺发育不全2例病例报道及文献回顾
- Author:
Xue LI
1
;
Mengfan DING
;
Fei TENG
;
Yanfang ZHANG
;
Ying LUO
;
Yongxiu ZHENG
;
Jin CUI
;
Huiying ZHANG
Author Information
1. 天津医科大学总医院妇产科，天津 300052
- Keywords:
46XY simple gonadal hypoplasia;
Sweyer syndrome;
Primary amenorrhea
- From:
Clinical Medicine of China
2024;40(1):65-69
- CountryChina
- Language:Chinese
-
Abstract:
46XY simple gonadal hypoplasia, also known as Sweyer syndrome, patients often due to primary amenorrhea or pubertal secondary sex characteristics do not develop the doctor, its combined gonadal tumor is more likely, in the treatment process is often recommended prophylactic removal of gonads, postoperative hormone replacement therapy. We describe two patients diagnosed with Sweyer syndrome, one with gonadowlastoma and mature teratoma, and one with nodular Leydig cell hyperplasia and ectopic adrenal tissue, and reviews the literature.
