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June 1986

Picture of the Month

Author Affiliations

Contributed from the Department of Pediatrics, Spartanburg (SC) General Hospital.

Am J Dis Child. 1986;140(6):565-566. doi:10.1001/archpedi.1986.02140200075031

Denouement and Discussion 

Testicular Feminization Syndrome 

Manifestations  The testicular feminization syndrome is one of many causes of a suprapubic mass in a phenotypically female infant. The groin mass (testes) may be bilateral or unilateral. Patients are phenotypically female and have an XY karyotype, an absent clitoris, and a blind-ending vaginal pouch. Because of the lack of an active intracellular binding protein, androgen resistance occurs, which is manifested by the inability of an androgen-sensitive receptor to utilize testosterone and dihydrotestosterone. This results in lack of masculinization of the fetus in utero. Müllerian structures (fallopian tubes, uterus, and the upper third of the vagina) are absent because testicular Sertoli's cells are present and produce müllerian inhibitory factor. There are also no wolffian structures (seminal vesicles, vasa deferentia, and epididymides) because of androgen resistance.