Management of Gonads in Adults with Androgen Insensitivity: An International Survey.

Tack, Lloyd J W; Maris, Ellen; Looijenga, Leendert H J; Hannema, Sabine E; Audi, Laura; Köhler, Birgit; Holterhus, Paul-Martin; Riedl, Stefan; Wisniewski, Amy; Flück Pandey, Christa Emma; Davies, Justin H; T Apos Sjoen, Guy; Lucas-Herald, Angela K; Evliyaoglu, Olcay; Krone, Nils; Iotova, Violeta; Marginean, Otilia; Balsamo, Antonio; Verkauskas, Gilvydas; Weintrob, Naomi; ... (2018). Management of Gonads in Adults with Androgen Insensitivity: An International Survey. Hormone research in paediatrics, 90(4), pp. 236-246. Karger 10.1159/000493645

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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. 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. METHODS A survey was performed among health care professionals who use the International DSD Registry (I-DSD). 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. 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.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine

UniBE Contributor:

Flück Pandey, Christa Emma


600 Technology > 610 Medicine & health








Christa Emma Flück Pandey

Date Deposited:

26 Feb 2019 14:18

Last Modified:

26 Feb 2019 14:18

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PubMed ID:


Uncontrolled Keywords:

Complete androgen insensitivity syndrome DSD management Disorders of Sex Development (DSD) Germ cell cancer International DSD Registry Partial androgen insensitivity syndrome


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