46, XX Male or Ovotesticular DSD (SRY-negative) without SRY, is it Possible to have Testicular Tissues?

Abstract

The 46, XX male syndrome, or the 46, XX testicular Disorder of Sexual Differentiation (DSD) is rare. We report a clinical case of a male infant 46, XX, DSD with SRY negative. This observation suggests that genes other than SRY play an important role in determining sex. From this case we will present a review of the literature to take stock of the subject.