SAEM Clinical Image Series: Inguinal Masses

DDX: Bilateral inguinal hernias, abscess, undescended testicles.

The abnormal findings in this patient were consistent with undescended rudimentary testicles in a patient without a prior diagnosis of true hermaphroditism (an ovotesticular disorder of sex development [DSD]). Our patient was phenotypically a female, with developed breasts, vagina, uterus, and ovaries, although uterus and ovaries were noted to be diminutive on imaging. Our patient identified herself as a female of homosexual orientation.

True hermaphroditism is characterized as the presence of seminiferous tubules and ovarian follicles in the same individual. Genitalia is usually ambiguous; however, patients may appear phenotypically male or female. Reproductive organ differentiation is a complex process involving multiple pathways and receptors, predominantly antimüllerian hormone (AMH). When testes differentiate, the secretion of AMH will lead to regression of müllerian structures, and lack of AMH will result in progression of female organ development (e.g., fallopian tubes, uterus, cervix, and upper vagina). The vast majority of patients with hermaphroditism will have a uterus or uterine horn, and most patients have breast development, ovulation, and menstruation.