Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.
OBJECTIVE
Adult women with complete androgen insensitivity syndrome (CAIS) are increasingly likely to defer or decline gonadectomy despite counselling about malignancy risk. The objectives of this study were to review the evidence on the risk of gonadal malignancy in adult women with CAIS and to explore women's reasons for deferring gonadectomy.


METHODS
A case series and literature review.


METHODS
Sixteen women with CAIS over the age of 18 years who have elected to defer gonadectomy.


RESULTS
Sixty-two relevant papers were identified. Of these, 14 confirmed that tumours had been reported in 98 adults. Taking into account the limitations of combining historic case series, this review estimates a risk of gonadal malignancy of 14% (range 0% and 22%) in adults with CAIS. The most common reasons women offered for deferring gonadectomy included inconvenience of surgery, concern about surgical risk and reluctance to take hormone replacement therapy.


CONCLUSIONS
Perceived benefits for retaining gonads in women with CAIS are prompting more women to keep their gonads in situ. An accurate estimate for adult malignancy risk is unavailable, and the risks currently quoted may be falsely reassuring.
University of New South Wales and Royal Hospital for Women, Sydney Australia, NSW, Australia.
Rebecca
R
Deans
Sarah M
SM
Creighton
Lih-Mei
LM
Liao
Gerard S
GS
Conway
D000293:Adolescent; D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000601:surgery*; D005260:Female; D006066:Gonads / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D055815:Young Adult