Hypertension and virilization caused by a unique desoxycorticosterone- andandrogen-secreting adrenal adenoma

Citation
D. Soranno et al., Hypertension and virilization caused by a unique desoxycorticosterone- andandrogen-secreting adrenal adenoma, J PED END M, 12(2), 1999, pp. 215-220
Citations number
18
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
ISSN journal
0334-018X → ACNP
Volume
12
Issue
2
Year of publication
1999
Pages
215 - 220
Database
ISI
SICI code
0334-018X(199903/04)12:2<215:HAVCBA>2.0.ZU;2-3
Abstract
We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrena l tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorr hea, hypertension and virilization. Her steroid pattern simulated an 11 bet a-hydroxylation defect with notable elevation of adrenal androgens, 11-deso xycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogeno us ACTH stimulated steroido-genesis. A CAT scan unfortunately failed to del ineate an adrenal mass. Dexamethasone (DEX) was administered, therefore, wh ich partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a rig ht adrenal tumor, Post surgery, the patient promptly resumed menses and bec ame normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of Delta(4) and Delta( 5) steroids, present here, may favor a tumor. This case also allows specula tion that the hypersecretion of DOC may result from inhibition of 11 beta-h ydroxylase activity by excess androgens, The importance of appropriate imag ing for diagnosis is underscored.