Abdominal Mass in a Phenotypic Female with 46,XY Differences in Sex Development

We present a case of a large intra-abdominal mass found to be localized pure seminoma within a retained gonad of a 53 -year-old phenotypic female with 46,XY differences in sex development (DSD) and androgen insensitivity syndrome (AIS). Our management included extirpation of the mass with contralateral gonadectomy. Historically, patients with AIS would undergo gonadectomy to mitigate the lifetime risk of testicular germ cell tumor development; however, grow-ing evidence suggests safety in retention and surveillance of these gonads into adulthood. This case highlights the impor-tance of lifetime surveillance of patients with 46,XY DSD who elect to retain their gonads. UROLOGY 173: e13-e16, 2023. (c) 2022 Elsevier Inc.

Automated summary

We reported a case of a 53-year-old phenotypic female with 46,XY differences in sex development (DSD) and androgen insensitivity syndrome (AIS) who had a large intra-abdominal mass identified as localized pure seminoma within a retained gonad. Our management approach included removal of the mass and contralateral gonadectomy. This case highlights the importance of lifetime surveillance in patients with 46,XY DSD who choose to retain their gonads.