Choukair, Daniela; Beuschlein, Felix; Zwermann, Oliver; Wudy, Stefan A.; Haufe, Sabine; Holland-Cunz, Stefan; Bettendorf, Markus
Virilization of a Young Girl Caused by Concomitant Ectopic and Intra-Adrenal Adenomas of the Adrenal Cortex.
In: Hormone Research in Paediatrics, No. 5: pp. 318-322
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.