A Testosterone-Secreting Tumour of the Adrenal Producing Virilisation in a Female Infant

A Testosterone-Producing Adrenal Cortical Adenoma in an Elderly Woman

“…Embryologically the adrenal blastema lies in close apposition to the genital ridge. The rare presence of ovarian thecal metaplasia within the adrenal may be explained by true ectopia of normally developed tissue or by a metaplasia of embryologically competent mesenchymal cells subjected to extended stimulation by elevated levels of gonadotropins (15,16). Although the histology of the tumor would suggest nothing more than an adrenal adenoma, it is possible that this tumor arose from mesenchymal cells with the potential of synthesizing testosterone, and underwent metaplasia when stimulated by the high gonadotropin levels in this patient.…”

“…Retrograde venous sampling has been utilized to locate endocrine active neoplasms (22,23), including a virilizing ovarian tumor (24). Although retrograde venous sampling was unsuccessful at localizing a testosterone-producing adrenal tumor in a 20-month-old girl (16), a technique led successfully to the accurate preoperative diagnosis of a virilizing adrenal tumor in this patient. This case demonstrates again that the use of retrograde venous sampling of ovarian and adrenal veins may locate effectively the source of excess androgens in virilized patients in whom the diagnosis is not obvious.…”

Testosterone-Producing Adrenocortical Neoplasm in a 6-Year-Old Boy

“…Our patient is the youngest boy described in the literature with this rare hormonal constellation. The only younger similar patients were two girls, ages 20 and 24 months (4,13).…”

“…Adolescent girls may demonstrate menstrual abnormalities (6). Features of Cushing syndrome are found in over one quarter of all cases, and aldosterone or aldosterone-like products secreted by the tumour may cause elevated blood pressure and electrolyte abnormalities (1,4,15).…”

“…They most commonly present with virilisation in girls and pseudoprecocious puberty in boys 7, accompanied by Cushing syndrome in one quarter of paediatric patients (11). A small minority of these tumours predominantly secrete testosterone without significantly elevated urinary steroid levels (4,8,13).…”