Abstract
Background: Patients with 46,XY differences of sex development (DSD) due to 17β-hydroxysteroid dehydrogenase type 3 (17βHSD3) deficiency are generally assigned female at birth. With known future virilizing puberty, orchiectomy is often discussed with patients and their families. Decision-making and factors to consider around orchiectomy are complex and challenging. We report two patients who presented at different ages to highlight the considerations and complexities surrounding gonad management, including the likelihood of gonadal malignancy, the possibility of fertility and options for preservation, and the current knowledge of gender identity outcomes.
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