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

Horm Res Paediatr. 2018 Oct 18:1-11. doi: 10.1159/000493645. [Epub ahead of print]

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

Tack LJW1, Maris E2, Looijenga LHJ3, Hannema SE4,5, Audi L6, Köhler B7, Holterhus PM8, Riedl S9,10, Wisniewski A11, Flück CE12, Davies JH13, T Apos Sjoen G14, Lucas-Herald AK15, Evliyaoglu O16, Krone N17, Iotova V18, Marginean O19, Balsamo A20, Verkauskas G21, Weintrob N22, Ellaithi M23, Nordenström A24, Verrijn Stuart A25, Kluivers KB26, Wolffenbuttel KP27, Ahmed SF28, Cools M1.

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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.


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.


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


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.


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.

© 2018 S. Karger AG, Basel.


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