性腺母细胞瘤在一名遗传女性中表现为同性性早熟。

Gonadoblastoma presenting as isosexual precocious puberty in a genetic female.

作者信息

Steinhardt G F, Ine M Y, Subramanian M G, Hukku B, Perrin E V, Hoffman W H

出版信息

J Pediatr Surg. 1986 Oct;21(10):905-7. doi: 10.1016/s0022-3468(86)80022-7.

Abstract

A 6 1/2-year-old female with pseudoprecocious puberty was found to have a gonadoblastoma. Her karyotype was 46, XX; serum estradiol level was markedly elevated; and androgens were within the range of normal. The importance of the Y chromosome and dysgenetic gonad in the predisposition to development of a gonadoblastoma is reviewed. A further categorization of gonadoblastomas based on the presence or absence of the Y chromosome is suggested.

摘要

一名6岁半的假性性早熟女性被发现患有性腺母细胞瘤。她的核型为46,XX；血清雌二醇水平显著升高；雄激素水平在正常范围内。本文回顾了Y染色体和发育不全性腺在性腺母细胞瘤发生易感性中的重要性。建议根据Y染色体的有无对性腺母细胞瘤进行进一步分类。