Helpap B, Schwinger E, Spiertz K
Geburtshilfe Frauenheilkd. 1980 Apr;40(4):381-4. doi: 10.1055/s-2008-1039326.
By modern cytogenetic methods gonadal dysgenese are more often and exactly clarified than in former times. The cytogenetic analyses have shown that pure XY gonadal dystenesis is often combined with germ cell tumors. A hypergonadotropic ovarian insufficiency as well as a rapidly growing tumor of the left adnexa were diagnosed in a 26-year old woman. At abdominal hysterectomy and bilateral adnexectomy a dysgerminoma on the left side was found as well as on the right side gonadal stroma and rudiments of the Wolffian duct. Blood lymphocytes showed a 46, XY karyotype. The H-Y antigen determination was a positive intermediary titer as compared with normal female and male controls. The cytogenetic demonstration of a pure gonadal dysgenesis in patients with dysgerminoma is important because bilateral development of tumors may occur. Therefore, hysterectomy with bilateral adnexectomy should be carried out.
通过现代细胞遗传学方法,性腺发育不全比以前更常被准确地阐明。细胞遗传学分析表明,单纯XY性腺发育不全常与生殖细胞肿瘤相关。一名26岁女性被诊断为高促性腺激素性卵巢功能不全以及左侧附件快速生长的肿瘤。在腹部子宫切除术和双侧附件切除术中,发现左侧为无性细胞瘤,右侧为性腺间质和中肾管残迹。血液淋巴细胞显示46, XY核型。与正常女性和男性对照相比,H-Y抗原测定为阳性中间滴度。无性细胞瘤患者中单纯性腺发育不全的细胞遗传学证明很重要,因为可能发生双侧肿瘤。因此,应进行子宫切除术加双侧附件切除术。