Gonadal dysgenesis in disorders of sex development: Diagnosis and surgical management

被引：42

作者：

Wolffenbuttel, K. P. ^{[1
]}

Hersmus, R. ^{[2
]}

Stoop, H. ^{[2
]}

Biermann, K. ^{[2
]}

Hoebeke, P. ^{[3
]}

Cools, M. ^{[4
]}

Looijenga, L. H. J. ^{[2
]}

机构：

[1] Erasmus MC, Sophia Childrens Hosp, Dept Urol & Pediat Urol, Room Sk-1270,POB 2060, NL-3000 CB Rotterdam, Netherlands

[2] Erasmus MC, Josephine Nefkens Inst, Dept Pathol, Rotterdam, Netherlands

[3] Ghent Univ Hosp, Dept Urol & Pediat Urol, Ghent, Belgium

[4] Univ Ghent, Ghent Univ Hosp, Dept Pediat, B-9000 Ghent, Belgium

来源：

JOURNAL OF PEDIATRIC UROLOGY | 2016年 / 12卷 / 06期

关键词：

Gonadal dysgenesis (GD); Malignant germ cell tumors; Germ cell cancer (GCC); Disorders of sex development (DSD); Surgical management; ANDROGEN INSENSITIVITY SYNDROME; GERM-CELL NEOPLASIA; CARCINOMA IN-SITU; GONADOBLASTOMA; EXPRESSION; TUMORS; WOMEN; GENE;

D O I：

10.1016/j.jpurol.2016.08.015

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Recent studies on gonadal histology have improved the understanding of germ cell malignancy risk in patients with disorders of sex development (DSD), and evidence-based gonadal management strategies are gradually emerging. Especially in 46, XY DSD and 45, X/46, XY DSD, which are characterized by gonadal dysgenesis, the risk of germ cell malignancy is significantly increased. This paper summarized the progress over the past 10 years in malignancy risk assessment in patients with DSD, and its implications for optimal surgical handling of the involved gonads.

引用

页码：411 / 416

页数：6

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