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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。
This article reports one patient with a 45, XO/47,XYY meioblastoma karyotype, combined with 5 cases of hemoblastoma reported in 7 cases of this type of karyotype in the past 30 years. The incidence of meioblastoma in this type of karyotype is 75% (6/8). The karyotype analysis of one case of gonadal tissue culture in this hospital showed that the tumor side XYY cell line was dominant (80%), while the non-tumor side accounted for only 11%. The histological origin of the gonad in 6 tumors, except for one case that developed in the hypoplastic testis, came from cord-like gonads. The clinical manifestations of these cases are related to histological histology and sex hormones secreted by the tumor. In patients with Turner signs, karyotype analysis should be performed with or without the development of sexual characteristics. If there are Y chromosomes, gonads should be removed as soon as possible to avoid tumors.