Management of males with 45,X/46,XY gonadal dysgenesis

Management of males with 45,X/46,XY gonadal dysgenesis
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DOI:
10.1159/000023425
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发表时间:
1999-07-01
期刊:
影响因子:
--
通讯作者:
Skakkebæk, NE
Skakkebæk, NE
中科院分区:
其他
文献类型:
--
作者:
Müller, J;Ritzén, EM;Skakkebæk, NE

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45、X/46、XY核型和外生殖器畸形的男性生殖腺生殖细胞瘤发生的风险增加。我们研究了10名年龄在0.3-17岁的男性患者的性腺组织,这些患者要么是尿道下裂,要么是隐睾。4例患者,年龄0.3-15岁,有原位癌,1例男孩有支持细胞模式,其余青春期前组织学,性腺母细胞瘤或浸润性癌未发现。在我们目前的知识的基础上,我们提出了一个策略,以管理和随访这些男孩为了发现可能的癌前组织学变化和早期预防性腺肿瘤的发展。版权所有(C) 2000 S. Karger AG,巴塞尔。
Males with the 45,X/46,XY karyotype and malformations of the external genitalia carry an increased risk of developing germ cell neoplasia of the gonads. We have studied gonadal tissue from 10 individuals, 0.3-17 years of age, with a male phenotype and either hypospadias and/ or cryptorchidism. Four patients, 0.3-15 years of age, had carcinoma in situ, 1 boy had Sertoli-cell-only pattern and the remainder prepubertal histology, Gonadoblastoma or invasive carcinoma was not found. On the basis of our current knowledge we propose a strategy for management and follow-up of these boys in order to detect possible premalignant histological changes early and prevent development of a gonadal tumour. Copyright (C) 2000 S. Karger AG, Basel.