Virilization of a Young Girl Caused by Concomitant Ectopic and Intra-Adrenal Adenomas of the Adrenal Cortex

Background: Adenomas of the adrenal gland are rare causes
ofvirilization in childhood. Case Report: A girl aged 2 years and 4
monthspresented with pubarche, distinct clitoral hypertrophy, tall
stature,and increased height velocity. Plasma testosterone
anddehydroepiandrosterone were elevated. Androgens remained
unchanged afteradrenocorticotropic hormone, and dexamethasone
administrations.Ultrasound examination and magnetic resonance
imaging indicated anextra-adrenal mass adjacent to the left adrenal
gland, which was removedby endoscopic surgery. However, plasma
androgens remained elevated and131 I-iodomethyl-norcholesterol
scintigraphy revealed tracer enhancementin the right adrenal gland,
which was consecutively removed.Virilization regressed after
extirpation of the adenomas and heightvelocity normalized. Results:
Histology revealed a circumscribed adenomain the right adrenal
gland and an epithelial mass with adrenal corticalcells in the
left-sided ectopic tumor. In the ectopic tumor,melanocortin 2
receptor expression was augmented threefold compared tothe control,
indicating adrenal origin. Conclusions: In this young
girl,virilization is due to concomitant ectopic and intra-adrenal
adenomas ofthe adrenal cortex. By melanocortin 2 receptor
expression, it wasconfirmed that the ectopic adenoma derived from
the adrenal cortex.Specific scintigraphy, if available, assists in
allocating the source ofandrogen hypersecretion.