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

Abstract 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...

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Published inJournal of pediatric urology Vol. 12; no. 6; pp. 411 - 416
Main Authors Wolffenbuttel, K.P, Hersmus, R, Stoop, H, Biermann, K, Hoebeke, P, Cools, M, Looijenga, L.H.J
Format Journal Article
LanguageEnglish
Published England Elsevier Ltd 01.12.2016
Subjects
Child
Decision Trees
Disorders of sex development (DSD)
Female
Germ cell cancer (GCC)
Gonadal dysgenesis (GD)
Gonadal Dysgenesis - diagnosis
Gonadal Dysgenesis - surgery
Humans
Male
Malignant germ cell tumors
Pediatrics
Surgical management
Urologic Surgical Procedures - methods
Urology
Surgical management
Gonadal dysgenesis (GD)
Disorders of sex development (DSD)
Malignant germ cell tumors
Germ cell cancer (GCC)
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Summary:Abstract 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.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
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ObjectType-Review-1
ISSN:1477-5131
1873-4898
DOI:10.1016/j.jpurol.2016.08.015