Hypertension and virilization caused by a unique desoxycorticosterone- and androgen-secreting adrenal adenoma.
We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. Dexamethasone (DEX) was administered, therefore, which 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 right adrenal tumor. Post surgery, the patient promptly resumed menses and became 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 speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored.
Department of Pediatrics, New York University Medical Center, NY 10016, USA.
D
D
Soranno
V
V
Prasad
R
R
David
S
S
Oberfield
A
A
Greco
N
N
Sivaraman
W
W
Drucker
D000236:Adenoma / Q000097:blood / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D000324:Adrenocorticotropic Hormone; D000728:Androgens / Q000378:metabolism*; D003900:Desoxycorticosterone / Q000378:metabolism*; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006889:Hydroxycorticosteroids / Q000097:blood; D006908:Hydroxyprogesterones / Q000097:blood; D006973:Hypertension / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D014770:Virilism / Q000097:blood / Q000188:drug therapy / Q000209:etiology*
D000728:Androgens; D006889:Hydroxycorticosteroids; D006908:Hydroxyprogesterones; D003900:Desoxycorticosterone; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone