Gonadal dysgenesis in disorders of sex development: Diagnosis and surgical management. Issue 6 (December 2016)

• Record Type: Journal Article
• Authors: Wolffenbuttel, K.P.; Hersmus, R.; Stoop, H.; Biermann, K.; Hoebeke, P.; Cools, M.; Looijenga, L.H.J.
• Is Part Of: Journal of pediatric urology. Volume 12:Issue 6(2016)
• Page Start: 411
• Page End: 416
• Publication Date: 2016-12
• Subjects: Gonadal dysgenesis (GD) -- Malignant germ cell tumors -- Germ cell cancer (GCC) -- Disorders of sex development (DSD) -- Surgical management; Pediatric urology -- Periodicals; Urologic Diseases -- Periodicals; Urogenital Diseases -- Periodicals; Urologic Surgical Procedures -- Periodicals; Child; Infant; Urologie pédiatrique -- Périodiques; Appareil urinaire -- Maladies -- Périodiques; Pédiatrie; Urologie; Pediatric urology; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); Electronic journals; Periodicals; Electronic journals; 618.926
• DOI: 10.1016/j.jpurol.2016.08.015
• Languages: English
• ISSNs: 1477-5131

Abstract

Summary: 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.