Abstract

Objective: To report the rare case of a patient with Complete Androgen Insensitivity Syndrome (CAIS) with Klinefelter Syndrome (KS) (47, XXY) presenting a Sertoli/Leydig cell tumor discovered during the gonadectomy.

Design: Single case report.

Setting: Academic clinic.

Patient(s): Patient with 36years-old seeking assistance with decreased libido.

Intervention(s): Adequate hormone replacement therapy, which had not previously been instituted.

Result(s): The patient had improved secondary sexual characteristics but only partially improved libido.

Conclusion(s): The clinical case described shows a rare phenomenon such as the association between CAIS and KS, associated with the finding of Sertoli/Leydig cell tumor after gonadectomy.

Keywords

androgen insensitivity syndrome, klinefelter syndrome, sertoli/leydig cell tumor, hormone replacement therapy, endocrinology