Gonadectomy in conditions affecting sex development: a registry-based cohort study. Issue 6 (4th May 2021)

• Record Type: Journal Article
• Authors: Lucas-Herald, Angela K; Bryce, Jillian; Kyriakou, Andreas; Ljubicic, Marie Lindhardt; Arlt, Wiebke; Audi, Laura; Balsamo, Antonio; Baronio, Federico; Bertelloni, Silvano; Bettendorf, Markus; Brooke, Antonia; Claahsen van der Grinten, Hedi L; Davies, Justin H; Hermann, Gloria; de Vries, Liat; Hughes, Ieuan A; Tadokoro-Cuccaro, Rieko; Darendeliler, Feyza; Poyrazoglu, Sukran; Ellaithi, Mona; Evliyaoglu, Olcay; Fica, Simone; Nedelea, Lavinia; Gawlik, Aneta; Globa, Evgenia; Zelinska, Nataliya; Guran, Tulay; Güven, Ayla; Hannema, Sabine E; Hiort, Olaf; Holterhus, Paul-Martin; Iotova, Violeta; Mladenov, Vilhelm; Jain, Vandana; Sharma, Rajni; Jennane, Farida; Johnston, Colin; Guerra Junior, Gil; Konrad, Daniel; Gaisl, Odile; Krone, Nils; Krone, Ruth; Lachlan, Katherine; Li, Dejun; Lichiardopol, Corina; Lisa, Lidka; Markosyan, Renata; Mazen, Inas; Mohnike, Klaus; Niedziela, Marek; Nordenstrom, Anna; Rey, Rodolfo; Skaeil, Mars; Tack, Lloyd J W; Tomlinson, Jeremy; Weintrob, Naomi; Cools, Martine; Ahmed, S Faisal;
• Is Part Of: European journal of endocrinology. Volume 184:Issue 6(2021)
• Page Start: 791
• Page End: 801
• Publication Date: 2021-05-04
• Subjects: Endocrinology -- Periodicals; 616.4005
• DOI: 10.1530/EJE-20-1058
• Languages: English
• ISSNs: 0804-4643

Abstract

Abstract : Objectives: To determine trends in clinical practice for individuals with DSD requiring gonadectomy. Design: Retrospective cohort study. Methods: Information regarding age at gonadectomy according to diagnosis; reported sex; time of presentation to specialist centre; and location of centre from cases reported to the International DSD Registry and who were over 16 years old in January 2019. Results: Data regarding gonadectomy were available in 668 (88%) individuals from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cases (53%). Females were more likely to undergo gonadectomy ( n = 311, P < 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high-income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), respectively ( P < 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries. Conclusions: The likelihood of gonadectomy depends on the underlying diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy.