摘要
本文报道1例核型为45,XO/47,XYY的性母细胞瘤,结合近30年文献中该类核型7例报道中发生性母细胞瘤的5例。性母细胞瘤在该类核型的发生率为75％(6/8)。本院1例性腺组织培养的核型分析显示肿瘤侧XYY细胞系占优势(80％),而非肿瘤侧仅占11％。6例肿瘤的性腺组织学来源,除1例发生于发育不全的睾丸外,均来自条索状性腺。这些病例的临床表现与性腺组织学及肿瘤分泌的性激素有关。具Turner体征的患者,伴或不伴性征发育均应做核型分析,如果有Y染色体存在,应尽早切除性腺以免发生肿瘤。
A case of gonadoblastoma with 45, XO/47, XYY mosaicism is reported. Another 7 cases of the same mosaicism in the literature during the last 30 years are reviewed. Including our case, gonadoblastoma occurred in 6 of the 8 cases. Gonadal tissue culture of our case showed an incidence of XYY-cells from the gonad containing the tumor to be 80%, whereas on the contralateral non-tumorous side, the incidence was 11 %. All tumors, except one from a dysgenetic testicle, have been found to occur in streak gonads. Clinical manifestations of the cases are related to the gonadal histology and sex steroid hormones secreted by the gonadoblastoma. Cases manifesting Turner's syndrome with or with out sexal development should be karyotyped. If there is the presence of the Y chromosome or chromosomes, gonadectomy should be done early to avoid tumorous development.
出处
《上海医科大学学报》
CSCD
1991年第5期379-382,共4页
Journal of Fudan University(Medical Science)
关键词
性母细胞瘤
性腺肿瘤
嵌合体
gonadoblastoma
45, XO/47, XYY mosaicism
gonadal dysgenesis
