A Case of True Hermaphroditism Presenting as a Testicular Tumour

Abstract

True hermaphroditism represents only 5% cases of all of disorders of sexual differentiation (DSD) and usually present in early childhood with ambiguous genitalia. Occasionally, cases might present later on in adolescence with problems of sexual maturation. Our case report presents a true hermaphrodite with normal male phenotype that presented as a left testicular mass, two years after being diagnosed with Sertoli cell only syndrome in the contralateral testis. Histological examination of the left testis showed ovarian, fallopian tube, myometrial, endometrial, and epididymal tissue. This combination of findings is found in approximately one-third of true hermaphrodites, but it is very rare to present clinically as an inguinoscrotal mass.

An ovulating follicle presenting as a testicular mass in a teenage patient with ovotesticular DSD

“…A 22-year old phenotypic male presenting with an intratesticular mass comprised of ovarian tissue has been reported. 5 Our case is truly unique as our patient had histologically documented Alternating ODSD as a newborn. Despite the apparent absence of ovarian tissue in the descended gonad, he was later found to have an ovarian remnant when the ovarian tissue formed a corpus luteum cyst, thus changing his classification to Unilateral ODSD.…”

“…Not surprisingly, Wolffian structures are noted with a testicle and Mullerian structures next to an ovary the majority of the time. 5 …”

Dysgerminoma in Pseudohermaphroditism: A Case Report

“…In a hermaphrodite, a tumor can arise either from ovarian tissue or testicular tissue, often the gonads are destroyed so that site of origin cannot be determined. 7 A 50% of the ovotestes are found in an abdominal position, while 25% are in the inguinal region.…”

“…8 The type of internal genitalia found depends on the adjacent gonads. 7 The gonads are at high risk for gonadal tumors, which are typically gonadoblastomas and/or dysgerminomas. 9,10 Dysgerminomas are generally rare, accounting for less than 5% of ovarian tumors, but exhibit a high malignant potential.…”

Analytical Review of Contemporary Fatwas in Resolving Biomedical Issues Over Gender Ambiguity

“…In classical Islamic literature, it is widely understood that khunthā are those who have dual genitalia or have nothing but an orifice for urination. Conversely, there is very little evidence in medical diagnostic of external dual genitalia cases (Sax August 2002 ; Ceci et al 2015 ) and the non-existence of external genitalia is unknown in the medical literature. Al-Sibāʿi and Al-Barr ( 1993 ) claim that we should leave the cases to the experts in the field, i.e.…”