Endocrine Abstracts (2011) 26 P651

Adrenal rest in a patient with late onset 21-hydroxylase deficiency

A Chronaiou, I Kostoglou-Athanassiou, A Michou, S Kotanoglou, G Vassiliou, K Tzioras, V Loi & A Karfi

Department of Endocrinology, Red Cross Hospital, Athens, Greece.

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.

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