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
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.