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

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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. 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. (C) 2018 S. Karger AG, Basel

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Chicago
Tack, Lloyd J. W., Ellen Maris, Leendert H. J. Looijenga, Sabine E. Hannema, Laura Audi, Birgit Koehler, Paul-Martin Holterhus, et al. 2018. “Management of Gonads in Adults with Androgen Insensitivity: An International Survey.” Hormone Research in Paediatrics 90 (4): 236–246.
APA
Tack, L. J. W., Maris, E., Looijenga, L. H. J., Hannema, S. E., Audi, L., Koehler, B., Holterhus, P.-M., et al. (2018). Management of Gonads in Adults with Androgen Insensitivity: An International Survey. HORMONE RESEARCH IN PAEDIATRICS, 90(4), 236–246.
Vancouver
1.
Tack LJW, Maris E, Looijenga LHJ, Hannema SE, Audi L, Koehler B, et al. Management of Gonads in Adults with Androgen Insensitivity: An International Survey. HORMONE RESEARCH IN PAEDIATRICS. 2018;90(4):236–46.
MLA
Tack, Lloyd J. W. et al. “Management of Gonads in Adults with Androgen Insensitivity: An International Survey.” HORMONE RESEARCH IN PAEDIATRICS 90.4 (2018): 236–246. Print.
@article{8607598,
  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. 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. (C) 2018 S. Karger AG, Basel},
  author       = {Tack, Lloyd J. W. and Maris, Ellen and Looijenga, Leendert H. J. and Hannema, Sabine E. and Audi, Laura and Koehler, Birgit and Holterhus, Paul-Martin and Riedl, Stefan and Wisniewski, Amy and Flueck, Christa E. and Davies, Justin H. and T'Sjoen, Guy and Lucas-Herald, Angela K. and Evliyaoglu, Olcay and Krone, Nils and Iotova, Violeta and Marginean, Otilia and Balsamo, Antonio and Verkauskas, Gilvydas and Weintrob, Naomi and Ellaithi, Mona and Nordenstrom, Anna and Stuart, Annemarie Verrijn and Kluivers, Kirsten B. and Wolffenbuttel, Katja P. and Ahmed, S. Faisal and Cools, Martine},
  issn         = {1663-2818},
  journal      = {HORMONE RESEARCH IN PAEDIATRICS},
  number       = {4},
  pages        = {236--246},
  title        = {Management of Gonads in Adults with Androgen Insensitivity: An International Survey},
  url          = {http://dx.doi.org/10.1159/000493645},
  volume       = {90},
  year         = {2018},
}

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