Autoimmune destruction of the adrenocortical cells is the most frequent form of Addi-sons disease, however modern imaging methods make possible to discover bilateral neoplastic laesions resulting in adrenal insufficiency more frequently than about twenty years ago. In our group of 315 patients with Addisons disease we diagnosed metastatic infiltrations of the adrenals in 18 cases and lymphoma of both adrenals in four cases (in sum 7%). Typical clinical signs, biochemical abnormalities and results of hormonal examinations (low cortisol, high ACTH levels, lack of adrenal reserve during ACTH stimulation) were present in majority of them. In six cases pre-Addisons disease was diagnosed, presenting with fatigability and increased ACTH concentrations.
A specific form of adrenal insufficiency has been observed in patients with adrenal cancer, treated with mitotane, an adrenolytic agent. In such cases higher replacement doses of hydrocortisone, than in classic Addisons disease, were necessary (quick conversion to inactive forms of steroids, increased transcortin levels). In our material of 194 patients with adrenal cancer mitotane was administered in a long-term therapy in 168 cases. In 18 out of 50 survivors it was possible to withdraw mitotane within four to five years of therapy, however in two patients a continuous adrenal insufficiency has been observed.
Conclusions: A relationship of adrenal insufficiency with oncology is evident however it is not a frequent finding.
03 - 07 May 2008
European Society of Endocrinology