Endocrine Abstracts

Androgen secreting adrenal tumor is a rare cause of androgen excess in women. There are only few case-reports of androgen producing adenoma in a patient with congenital adrenal hyperplasia.

Our patient, 61 year old woman was referred to our department for progressive hirsutism and androgenic alopecia. She did not mention neither excessive facial hair growth nor balding until age of 59 when rapidly progressive balding and overt hirsutism started. She has had three childbirths and three abortions in the past.

Laboratory evaluation showed markedly elevated levels of androgens and other adrenal steroids (testosterone 9.1 nmol/l ((upper limit 3,0), DHEA androstendion 82.67 nmol/l (upper limit 6,30), DHEA: 7.30; S_DHEA: 4,78 (mol/l (upper limit 3,70) and 17-OH progesteron: 228.00 nmol/l (upper limit 4,50)). ACTH as well as urinary free cortisol were normal. Physiological pattern of diurnal variability of cortisol secretion was preserved. In overnight low-dose dexamethason suppression test partial supressibility of elevated androgens was preserved.

Gynecological investigation did not show any pathology in her ovaries. On CT there were enlargement (tumors) of her adrenals (40×30×30 mm in the right gland and 30×25×20 mm in the left). We have performed scintigraphy with ¹³¹I-6β-iodomethyl-19-norcholesterol which showed enhanced accumulation of the substance in the right adrenal gland. Laparoscopic adrenalectomy of the right adrenal was performed. Histology proved cortical adenoma 40×30×20 mm with diffuse hyperplasia of the surrounding adrenal tissue.

There was a significant decrease in testosterone postoperatively (1.66 nmol/l) as well as of androstendion (6.21) and DHEAS (0.39). On the contrary 17-OHP remained elevated (129.26) and ACTH raised up to 229 ng/l. Genetic test for suspected congenital adrenal hyperplasia (21 OH def.) revealed compound heterozygosity for CAH. Treatment with corticosteroids, to supress the androgen overproduction from the left adrenal was commenced.

Conclusion: Androgen secreting adenoma is a rare cause of hyperandrogenism. In the presented case adenoma had developed in a patient with, so far undiagnosed, congenital adrenal hyperplasia.