SERTOLI-LEYDIG CELL HAMARTOMA IN A PATIENT WITH COMPLETE ANDROGEN INSENSITIVITY SYNDROME

Inclusion:Complete androgen insensitivity syndrome is characterized by a phenotypic female with a 46 XY karyotype ,female external genitalia, breast development, internal bilateral undescended testes, and absence of pubic and axiliary hair. Individuals with complete androgen syndrome are at risk for the development of gonadal tumors such as sertoli cell adenomas, tubuler hamartomas and leiomyoma.

Case Report: A 28-year-old female patient was admitted to the gynecology clinic with a primary amenorrhea. In the ultrasound examination, the absence of müllerian duct-derived organs and shallow vagina detected. In genetic examination ;46 XY karyotype was detected.Right and left gonadectomy was performed.Grossly ;Well-defined, solid, yellow-brown colored areas were observed in the sections of the two gonads.In addition, the cross-section of a gonad white-brown colored solid areas were observed .The microscopic findings showed nodules composed of unidentified tubule structures, lined with immature sertoli cells, and an increased number of leydig cells. Such lesions are considered hamartomas. Also an area with a desmin (+) leiomyoma nodule present.

Conclusion:We reported our rare case with literature.