Testicular Feminization Syndrome - A Rare Case Report

Abstract

Testicular feminization syndrome is a genetic disorder that makes XY fetuses insensitive/unresponsive to the actions of the androgen hormones .Instead, they are born looking externally like normal girls and internally, there is a short blind pouch vagina and no uterus, fallopian tubes or ovaries. There are testis in the abdomen or in the inguinal canal. The androgen insensitivity syndrome occurs in one out of 20000 births. It can be incomplete (various sexual ambiguities) or complete (the person appears to be a woman). In this case report, a married nulliparous female reported to Gynecology & obstetric dept. for treatment of primary infertility and had history of primary amenorrhea. Physical examination revealed normal external female genitalia with normal breast development (Tanner Stage-5) and absence of axillary and pubic hair. Bilateral adnexal masses were removed and sent for histopathological examination. On microscopic examination both the ovaries revealed seminiferous tubules lined only by the sertoli cells with no evidence of spermatogenesis. No ovarian tissue seen.