Germ cell neoplasms in three intersex patients with 46,XY karyotype

Germ cell neoplasms in three intersex patients with 46,XY karyotype
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三名 46,XY 核型双性人患者的生殖细胞肿瘤

DOI:
10.1007/bf02551299
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发表时间:
2006
影响因子:
2
通讯作者:
B. Jacobsen
B. Jacobsen
中科院分区:
医学4区
文献类型:
--
作者:
L. Petersen;K. Kock;B. Jacobsen

文献摘要

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本文报告3例两性人性腺母细胞瘤合并其他生殖细胞肿瘤,核型均为46,XY。一名11岁的患者是一个真正的雌雄同体,其他人,年龄分别为13岁和18岁,都有性腺发育不全。不同的临床过程已经注意到。2例性腺母细胞瘤合并无性细胞瘤,其中1例同时发现畸胎瘤,第3例性腺母细胞瘤转变为畸胎癌。2例青春期患者恶变较重。因此,我们建议早期和预防性的性腺切除术。
This report presents 3 cases with gonadoblastoma mixed with other germ cell tumours in intersex patients, all with a 46,XY, karyotype. One 11-year-old patient was a true hermaphrodite, the others, aged 13 and 18, respectively, had both gonadal dysgenesis. Different clinical courses have been noted. Two patients had gonadoblastoma combined with dysgerminoma; in one of them a teratoma was also found. In the third case gonadoblastoma was transformed into teratocarcinoma. The malignant degeneration was more serious in the 2 pubertal patients. Thus, we recommend early and prophylactic gonadectomy.