A Management Protocol for Gonad Preservation in Patients with Androgen Insensitivity Syndrome

J Pediatr Adolesc Gynecol. 2019 Dec;32(6):605-611. doi: 10.1016/j.jpag.2019.06.005. Epub 2019 Jun 21.


Historically, individuals with androgen insensitivity syndrome (AIS) were managed with removal of gonadal tissue at various ages to avert the risk of gonadal malignancy. Recently, clinical practice changed, with gonadectomy being postponed until late adolescence. Adolescents and adults with complete AIS have questioned this approach. Additionally, testicular germ cell tumors are increasingly believed to be quite rare with rates as low as 0% in molecularly confirmed individuals with AIS. Gonadectomy deprives patients of the benefits of their endogenous hormones and potential fertility. Furthermore, human rights organizations advocate for deferring irreversible surgery in conditions known as differences of sex development, which includes AIS, to allow patient autonomy in decision-making. Recent literature supports an approach that uses risk stratification to manage gonads in AIS. Herein we review what is known about malignancy risk in the different subtypes of AIS and propose a management protocol for gonad retention.

Keywords: Androgen insensitivity syndrome; Gonadectomy; Gonads; Malignancy risk.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Androgen-Insensitivity Syndrome / complications
  • Androgen-Insensitivity Syndrome / surgery*
  • Castration / standards*
  • Clinical Protocols / standards*
  • Female
  • Fertility Preservation / standards*
  • Gonads / surgery
  • Humans
  • Male
  • Neoplasms, Germ Cell and Embryonal / etiology
  • Neoplasms, Germ Cell and Embryonal / prevention & control
  • Sexual Development
  • Testicular Neoplasms / etiology
  • Testicular Neoplasms / prevention & control

Supplementary concepts

  • Testicular Germ Cell Tumor