Abstract
Two pediatric cases of XY females with normal female external genitalia and caused by different etiologies are presented.
Case 1 (2-months-old girl) was admitted for bilateral inguinal masses and measurement of serum testosterone levels suggested the diagnosis of infantile testicular feminization syndrome. Results of exploratory laparotomy and biopsy of the gonads confirmed the diag-nosis.
Case 2 (8-years-old girl) had the normal external genitalia without palpable gonadal masses. Serum levels of LH and FSH were elevated whereas serum testosterone level was low. Exploratory laparotomy revealed the presence of the uterus and fallopian tubes with bilateral gonadal masses which were removed. Histologically, the both gonads con-tained areas compatible with “bilateral gonadoblastoma occurring in XY-gonadal dysgenesis”.
