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Management of Gonads in Adults with Androgen Insensitivity: An International Survey.
- Source :
-
Hormone research in paediatrics [Horm Res Paediatr] 2018; Vol. 90 (4), pp. 236-246. Date of Electronic Publication: 2018 Oct 18. - Publication Year :
- 2018
-
Abstract
- Background: Complete and partial androgen insensitivity syndrome (CAIS, PAIS) are associated with an increased risk of gonadal germ cell cancer (GGCC). Recent guidelines recommend gonadectomy in women with CAIS in late adolescence. Nevertheless, many adult women prefer to retain their gonads.<br />Aims: This study aims to explore attitudes towards gonadectomy in AIS in centres around the world, estimate the proportion of adults with retained gonads and/or who developed GGCC, and explore reasons for declining gonadectomy.<br />Methods: A survey was performed among health care professionals who use the International DSD Registry (I-DSD).<br />Results: Data were provided from 22 centres in 16 countries on 166 women (CAIS) and 26 men (PAIS). In CAIS, gonadectomy was recommended in early adulthood in 67% of centres; 19/166 (11.4%) women refused gonadectomy. Among 142 women who had gonadectomy, evidence of germ cell neoplasm in situ (GCNIS), the precursor of GGCC, was reported in 2 (1.4%) out of 8 from whom pathology results were formally provided. Nine out of 26 men with PAIS (34.6%) had retained gonads; 11% of centres recommended routine gonadectomy in PAIS.<br />Conclusion: Although development of GGCC seems rare, gonadectomy after puberty is broadly recommended in CAIS; in PAIS this is more variable. Overall, our data reflect the need for evidence-based guidelines regarding prophylactic gonadectomy in AIS.<br /> (© 2018 S. Karger AG, Basel.)
- Subjects :
- Adolescent
Adult
Androgen-Insensitivity Syndrome surgery
Female
Humans
Male
Neoplasms, Germ Cell and Embryonal pathology
Neoplasms, Germ Cell and Embryonal prevention & control
Orchiectomy
Ovarian Neoplasms pathology
Ovarian Neoplasms prevention & control
Ovariectomy
Ovary surgery
Testicular Neoplasms pathology
Testicular Neoplasms prevention & control
Testis surgery
Androgen-Insensitivity Syndrome pathology
Ovary pathology
Registries
Testis pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1663-2826
- Volume :
- 90
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Hormone research in paediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 30336477
- Full Text :
- https://doi.org/10.1159/000493645