Adrenal tumor complicating untreated 21-hydroxylase deficiency in a 5 1/2-year-old boy.
OBJECTIVE
A 5 1/2-year-old boy presenting with virilization was diagnosed as having classic 21-hydroxylase deficiency complicated by an adrenal tumor. We attempted to document a reduction in the size of the tumor with glucocorticoid therapy.


METHODS
Case study.


METHODS
Referral center.


METHODS
Glucocorticoid therapy was instituted for congenital adrenal hyperplasia. Surgery for the adrenal mass was deferred, and the size of the mass was monitored by serial ultrasonography.


RESULTS
Baseline values of serum 17 alpha-hydroxyprogesterone (186.6 nmol/L) and testosterone (24.7 nmol/L [7.1 ng/mL]) were elevated. After instituting steroid treatment, 17 alpha-hydroxyprogesterone was suppressed (13.0 nmol/L), and testosterone remained undetectable on follow-up. However, the size of the mass increased during 6 months. Unilateral adrenalectomy performed at that time revealed a well-encapsulated adenoma in a hyperplastic gland.


CONCLUSIONS
Untreated classic congenital adrenal hyperplasia may be complicated by an adrenal tumor even at a young age. Suppression of adrenal androgens by glucocorticoid therapy was not accompanied by regression of the tumor in our patient.
Bhatia
V
V
Department of Endocrinology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, India.
Shukla
R
R
Mishra
S K
SK
Gupta
R K
RK
D000236:Adenoma / Q000150:complications* / Q000000981:diagnostic imaging; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging; D000311:Adrenal Glands / Q000000981:diagnostic imaging; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications; D002675:Child, Preschool; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography