Virilization of a young girl caused by concomitant ectopic and intra-adrenal adenomas of the adrenal cortex
<b><i>Background:</i></b> Adenomas of the adrenal gland are rare causes of virilization in childhood. <b><i>Case Report:</i></b> A girl aged 2 years and 4 months presented with pubarche, distinct clitoral hypertrophy, tall stature, and increased height...
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| Main Authors: | , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
May 25, 2013
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| In: |
Hormone research in paediatrics
Year: 2013, Volume: 79, Issue: 5, Pages: 318-322 |
| ISSN: | 1663-2826 |
| DOI: | 10.1159/000350244 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1159/000350244 Verlag, lizenzpflichtig, Volltext: https://www.karger.com/Article/FullText/350244 
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| Author Notes: | Daniela Choukair, Felix Beuschlein, Oliver Zwermann, Stefan A. Wudy, Sabine Haufe, Stefan Holland-Cunz, Markus Bettendorf |
| Summary: | <b><i>Background:</i></b> Adenomas of the adrenal gland are rare causes of virilization in childhood. <b><i>Case Report:</i></b> A girl aged 2 years and 4 months presented with pubarche, distinct clitoral hypertrophy, tall stature, and increased height velocity. Plasma testosterone and dehydroepiandrosterone were elevated. Androgens remained unchanged after adrenocorticotropic hormone, and dexamethasone administrations. Ultrasound examination and magnetic resonance imaging indicated an extra-adrenal mass adjacent to the left adrenal gland, which was removed by endoscopic surgery. However, plasma androgens remained elevated and <sup>131</sup>I-iodomethyl-norcholesterol scintigraphy revealed tracer enhancement in the right adrenal gland, which was consecutively removed. Virilization regressed after extirpation of the adenomas and height velocity normalized. <b><i>Results:</i></b> Histology revealed a circumscribed adenoma in the right adrenal gland and an epithelial mass with adrenal cortical cells in the left-sided ectopic tumor. In the ectopic tumor, melanocortin 2 receptor expression was augmented threefold compared to the control, indicating adrenal origin. <b><i>Conclusions:</i></b> In this young girl, virilization is due to concomitant ectopic and intra-adrenal adenomas of the adrenal cortex. By melanocortin 2 receptor expression, it was confirmed that the ectopic adenoma derived from the adrenal cortex. Specific scintigraphy, if available, assists in allocating the source of androgen hypersecretion. |
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| Item Description: | Gesehen am 16.04.2021 |
| Physical Description: | Online Resource |
| ISSN: | 1663-2826 |
| DOI: | 10.1159/000350244 |