Partial androgen insensitivity syndrome in a 68-year-old patient

Abstract

Partial androgen insensitivity syndrome (PAIS) is a form of disorders of sexual development. Besides the issues of gender assignment, the fate of gonads in these patients poses a challenging problem. Debate still remains on the need and/or timing of gonadectomy in either complete or partial androgen insensitivity syndromes. In this case report, we present a 68year-old patient who was raised as a woman, stayed married for 45 years and admitted to our endocrinology department with complaint of male type hair distribution after initial examination following move to a nursing home. Physical examination revealed no breast development, a phallus of 6 cm, labia majoras that include testes and a blind ending vagina. Chromosomal analysis confirmed 46,XY with intact SRY and AZF regions. Pelvic ultrasonography and magnetic resonance imaging results indicated testicular tissue in labia majoras in addition to a rudimentary prostate. Gonadectomy was not offered to the patient due to lacking evidence of benefit in this age group and considering possible hormonal side effects. Our patient might be the oldest patient to be diagnosed with PAIS. Treatment and follow-up protocols for adults with PAIS are not standardized and therefore these patients should be individually evaluated and treated. Risks and benefits of surgery should be kept in mind when suggesting gonadectomy.