Abstract
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.
Key Words
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Article info
Publication history
Published online: June 21, 2019
Footnotes
Erica M. Weidler is a paid board member and Executive Director of Accord Alliance. The remaining authors indicate no conflicts of interest.
Identification
Copyright
© 2019 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.
