title: 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 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.
authors: Department of Urology and Pediatric Urology, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: k.wolffenbuttel@erasmusmc.nl.
authors: K P
authors: KP
authors: Wolffenbuttel
authors: Department of Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, The Netherlands.
authors: R
authors: R
authors: Hersmus
authors: Department of Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, The Netherlands.
authors: H
authors: H
authors: Stoop
authors: Department of Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, The Netherlands.
authors: K
authors: K
authors: Biermann
authors: Department of Urology and Pediatric Urology, Ghent University Hospital, Ghent, Belgium.
authors: P
authors: P
authors: Hoebeke
authors: Department of Pediatrics, Ghent University Hospital, Ghent University, Belgium.
authors: M
authors: M
authors: Cools
authors: Department of Pathology, Erasmus MC, Josephine Nefkens Institute, Rotterdam, The Netherlands.
authors: L H J
authors: LH
authors: Looijenga
mesh_terms: D002648:Child; D003663:Decision Trees; D005260:Female; D006059:Gonadal Dysgenesis / Q000175:diagnosis* / Q000601:surgery*; D006801:Humans; D008297:Male; D013520:Urologic Surgical Procedures / Q000379:methods
keywords: Disorders of sex development (DSD); Germ cell cancer (GCC); Gonadal dysgenesis (GD); Malignant germ cell tumors; Surgical management