'Size does matter': Prophylactic gonadectomy in a case of Swyer syndrome.
Complete gonadal dygenesis
Dysgenetic gonads
Dysgerminoma
Gonadoblastoma
Immature teratoma
Swyer syndrome
Journal
Journal of gynecology obstetrics and human reproduction
ISSN: 2468-7847
Titre abrégé: J Gynecol Obstet Hum Reprod
Pays: France
ID NLM: 101701588
Informations de publication
Date de publication:
Apr 2019
Apr 2019
Historique:
received:
08
11
2018
revised:
22
01
2019
accepted:
23
01
2019
pubmed:
29
1
2019
medline:
3
8
2019
entrez:
29
1
2019
Statut:
ppublish
Résumé
Swyer syndrome also known as pure or complete gonadal dysgenesis is a very rare disorder of sex development wherein the individuals are phenotypically females with 46, XY genotype and preserved mullerian structures. These individuals characteristically have dysgenetic streak gonads which carry an increased risk of malignant transformation. Prophylactic gonadectomy is highly recommended as soon as a clinical diagnosis is established to diminish the chances of tumor development. We present a case of complete gonadal dysgenesis with bilateral small gonads with a dysgerminoma arising in a background of gonadoblastoma in one gonad and immature teratoma in the other. The present case, besides adding a rare case to the literature, highlights the importance of detailed pre-operative assessment of gonadal size and prompt prophylactic gonadectomy in cases with gonadal dysgenesis.
Identifiants
pubmed: 30690088
pii: S2468-7847(18)30481-1
doi: 10.1016/j.jogoh.2019.01.009
pii:
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Pagination
283-286Informations de copyright
Copyright © 2019 Elsevier Masson SAS. All rights reserved.