Adrenal rests, nodules of the adrenal, in patients with chronic elevation of ACTH levels have been previously described.
The aim was to describe the case of a patient with an adrenal tumor and late onset 21-hydroxylase deficiency.
A female patient, aged 76 years, presented with an incidentally discovered mass of the left adrenal measuring 1.2 cm. The patient was short with a height of 152 cm. Morning serum ACTH levels were 31 pg/ml, serum cortisol 16.8 μg/dl, serum aldosterone 14.7 pg/ml and serum renin 17 pg/ml, urine free cortisol 6.5 μg/day and 17(OH)progesterone 5 ng/ml. An intravenous ACTH stimulation test was performed. Cortisol levels before the administration of ACTH, 30 and 60 min later were 12, 13.7 and 14 μg/dl respectively. Serum 17(OH)progesterone levels before the administration of ACTH, 30 and 60 min later were 10, 50 and 50 ng/ml. The elevation of 17(OH)progesterone after the administration of ACTH was diagnostic of the presence of non classical 21-hydroxylase deficiency. The insufficient cortisol increase after the administration of ACTH was suggestive of partial adrenal insufficiency.
In conclusion, the rare case of a patient with non classical 21-hydroxylase deficiency is described. The patient presented in old age with an adrenal rest. It is possible that the chronic stimulation of the adrenal cortex from ACTH as a result of the partial cortisol insufficiency was involved in the pathogenesis of the adrenal tumor.
Rotterdam, The Netherlands
30 Apr - 04 May 2011
European Society of Endocrinology