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

Lloyd J W Tack; Ellen Maris; Leendert H J Looijenga; Sabine E Hannema; Laura Audi; Birgit Köhler; Paul-Martin Holterhus; Stefan Riedl; Amy Wisniewski; Christa E Flück; Justin H Davies; Guy T'Sjoen; Angela K Lucas-Herald; Olcay Evliyaoglu; Nils Krone; Violeta Iotova; Otilia Marginean; Antonio Balsamo; Gilvydas Verkauskas; Naomi Weintrob; Mona Ellaithi; Anna Nordenström; Annemarie Verrijn Stuart; Kirsten B Kluivers; Katja P Wolffenbuttel; S Faisal Ahmed; Martine Cools

doi:10.1159/000493645

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

Lloyd J W Tack, Ellen Maris, Leendert H J Looijenga, Sabine E Hannema, Laura Audi, Birgit Köhler, Paul-Martin Holterhus, Stefan Riedl, Amy Wisniewski, Christa E Flück, Justin H Davies, Guy T'Sjoen, Angela K Lucas-Herald, Olcay Evliyaoglu, Nils Krone, Violeta Iotova, Otilia Marginean, Antonio Balsamo, Gilvydas Verkauskas, Naomi WeintrobMona Ellaithi, Anna Nordenström, Annemarie Verrijn Stuart, Kirsten B Kluivers, Katja P Wolffenbuttel, S Faisal Ahmed, Martine Cools

Onderzoeksoutput: Bijdrage aan tijdschrift › Artikel › peer review

34 Citaten (Scopus)

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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.

Originele taal-2	Engels
Pagina's (van-tot)	236-246
Aantal pagina's	11
Tijdschrift	Hormone research in paediatrics
Volume	90
Nummer van het tijdschrift	4
DOI's	https://doi.org/10.1159/000493645
Status	Gepubliceerd - 2018
Extern gepubliceerd	Ja

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10.1159/000493645

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Tack, L. J. W., Maris, E., Looijenga, L. H. J., Hannema, S. E., Audi, L., Köhler, B., Holterhus, P.-M., Riedl, S., Wisniewski, A., Flück, C. E., Davies, J. H., T'Sjoen, G., Lucas-Herald, A. K., Evliyaoglu, O., Krone, N., Iotova, V., Marginean, O., Balsamo, A., Verkauskas, G., ... Cools, M. (2018). Management of Gonads in Adults with Androgen Insensitivity: An International Survey. Hormone research in paediatrics, 90(4), 236-246. https://doi.org/10.1159/000493645

@article{9f21b50036d644f4b0addd28fe861dc8,

title = "Management of Gonads in Adults with Androgen Insensitivity: An International Survey",

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.",

keywords = "Adolescent, Adult, Androgen-Insensitivity Syndrome/pathology, Female, Humans, Male, Neoplasms, Germ Cell and Embryonal/pathology, Orchiectomy, Ovarian Neoplasms/pathology, Ovariectomy, Ovary/pathology, Registries, Testicular Neoplasms/pathology, Testis/pathology",

author = "Tack, {Lloyd J W} and Ellen Maris and Looijenga, {Leendert H J} and Hannema, {Sabine E} and Laura Audi and Birgit K{\"o}hler and Paul-Martin Holterhus and Stefan Riedl and Amy Wisniewski and Fl{\"u}ck, {Christa E} and Davies, {Justin H} and Guy T'Sjoen and Lucas-Herald, {Angela K} and Olcay Evliyaoglu and Nils Krone and Violeta Iotova and Otilia Marginean and Antonio Balsamo and Gilvydas Verkauskas and Naomi Weintrob and Mona Ellaithi and Anna Nordenstr{\"o}m and {Verrijn Stuart}, Annemarie and Kluivers, {Kirsten B} and Wolffenbuttel, {Katja P} and Ahmed, {S Faisal} and Martine Cools",

note = "{\textcopyright} 2018 S. Karger AG, Basel.",

year = "2018",

doi = "10.1159/000493645",

language = "English",

volume = "90",

pages = "236--246",

journal = "Hormone research in paediatrics",

issn = "1663-2818",

publisher = "S. Karger AG",

number = "4",

}

Tack, LJW, Maris, E, Looijenga, LHJ, Hannema, SE, Audi, L, Köhler, B, Holterhus, P-M, Riedl, S, Wisniewski, A, Flück, CE, Davies, JH, T'Sjoen, G, Lucas-Herald, AK, Evliyaoglu, O, Krone, N, Iotova, V, Marginean, O, Balsamo, A, Verkauskas, G, Weintrob, N, Ellaithi, M, Nordenström, A, Verrijn Stuart, A, Kluivers, KB, Wolffenbuttel, KP, Ahmed, SF & Cools, M 2018, 'Management of Gonads in Adults with Androgen Insensitivity: An International Survey', Hormone research in paediatrics, vol. 90, nr. 4, blz. 236-246. https://doi.org/10.1159/000493645

TY - JOUR

T1 - Management of Gonads in Adults with Androgen Insensitivity

T2 - An International Survey

AU - Tack, Lloyd J W

AU - Maris, Ellen

AU - Looijenga, Leendert H J

AU - Hannema, Sabine E

AU - Audi, Laura

AU - Köhler, Birgit

AU - Holterhus, Paul-Martin

AU - Riedl, Stefan

AU - Wisniewski, Amy

AU - Flück, Christa E

AU - Davies, Justin H

AU - T'Sjoen, Guy

AU - Lucas-Herald, Angela K

AU - Evliyaoglu, Olcay

AU - Krone, Nils

AU - Iotova, Violeta

AU - Marginean, Otilia

AU - Balsamo, Antonio

AU - Verkauskas, Gilvydas

AU - Weintrob, Naomi

AU - Ellaithi, Mona

AU - Nordenström, Anna

AU - Verrijn Stuart, Annemarie

AU - Kluivers, Kirsten B

AU - Wolffenbuttel, Katja P

AU - Ahmed, S Faisal

AU - Cools, Martine

PY - 2018

Y1 - 2018

N2 - 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.

AB - 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.

KW - Adolescent

KW - Adult

KW - Androgen-Insensitivity Syndrome/pathology

KW - Female

KW - Humans

KW - Male

KW - Neoplasms, Germ Cell and Embryonal/pathology

KW - Orchiectomy

KW - Ovarian Neoplasms/pathology

KW - Ovariectomy

KW - Ovary/pathology

KW - Registries

KW - Testicular Neoplasms/pathology

KW - Testis/pathology

UR - http://www.scopus.com/inward/record.url?scp=85055458564&partnerID=8YFLogxK

U2 - 10.1159/000493645

DO - 10.1159/000493645

M3 - Article

C2 - 30336477

SN - 1663-2818

VL - 90

SP - 236

EP - 246

JO - Hormone research in paediatrics

JF - Hormone research in paediatrics

IS - 4

ER -

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

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