When a girl with Turner syndrome also carries Y chromosome material in some of her cells, an uncomfortable question surfaces early in the consultation: what should be done with her gonads? For years the answer was almost automatic — removal. A study published in Urology by a team from a multidisciplinary differences of sex development (DSD) clinic shows that the decision has become more nuanced: it now depends on each patient’s tumour-risk category, and in the Turner-with-Y group the authors themselves argue for talking it through with the family rather than defaulting to surgery.
The retrospective study reviews 81 patients seen between 2013 and 2024. The three most common diagnoses were Turner syndrome with Y chromosome material (23 patients), complete androgen insensitivity syndrome (15) and mixed gonadal dysgenesis (12). Each case was assigned to a gonadal tumour-risk category — high, intermediate or low — and the team compared what was done in each group and at what age.
The figures show a clear gradient. Surgery was performed in 96% of high-risk patients (24 of 25), 63% of intermediate-risk patients (20 of 32) and 38% of low-risk patients (9 of 24). Age differed too: median 6.7 years in the high-risk group, 9.7 years in the intermediate group and 16.4 years in the low-risk group. In other words, the higher the estimated risk, the earlier the operation; and in lower-risk cases teams tended to wait, sometimes until after puberty.
Tumours were found in nine patients: seven gonadoblastomas and two dysgerminomas. All occurred in the high- or intermediate-risk categories, and two-thirds arose in patients with complete gonadal dysgenesis, a particularly high-risk diagnosis. Malignant findings did not appear in the lowest-risk group; they clustered where anatomy and genetics had already raised suspicion.
For Turner syndrome with Y material, the largest subgroup in the series, the conclusion is explicit: the authors argue for case-by-case discussion instead of presenting removal as the default solution. In complete androgen insensitivity syndrome they propose delaying surgery until after puberty. And they stress that in high-risk diagnoses, above all complete gonadal dysgenesis, gonadectomy remains necessary.
The findings deserve a careful reading. This is a single centre, the patients carry very different diagnoses and the number of tumours is small, so it does not justify universal rules or looser follow-up. What it does offer is a useful framework for families: ask which risk category your daughter falls into, what surveillance is proposed in the meantime, which signals would call for earlier surgery and why a particular age is chosen for the operation. One further detail: across the study years neither the proportion of operations nor the age at surgery changed significantly, suggesting that the debate has yet to reshape everyday practice in every team.
Source: Gonadectomy Practices in a Multidisciplinary Differences of Sex Development Clinic Support a Risk-Stratified Approach. Khan HA, Talton A, Rosoklija I, et al. Urology 2026 (online publication). DOI: 10.1016/j.urology.2026.10.025.
Read the paper: https://doi.org/10.1016/j.urology.2026.10.025
