Although Cushing's disease is thought to be primarily a hypothalamic or pituitary disorder or both, therapy has traditionally been directed at the adrenal glands. Treatment by subtotal adrenalectomy has been largely abandoned because of the high rate of recurrence, approximately 30%.1 In view of the currently accepted mechanism of this disorder, any remaining adrenal tissue either left purposely or inadvertently would be exposed to continued stimulation by adrenocorticotropic hormone (ACTH). As a result, total bilateral adrenalectomy is the usual therapy at the present time, but because of technical surgical problems, or the presence of ectopic adrenal tissue, this procedure does not guarantee total remission. As far as we have been able to ascertain, there have been only four cases reported of recurrence after a presumed total adrenalectomy.2,3
We have recently observed four patients with recurrence or persistence of hypercortisolism following seemingly total adrenalectomy.
Following recognition of the
Siegal AM, Kreisberg RA, Hershman JM, Owen WC. Recurrent Cushing's Disease Following "Total" Adrenalectomy. Arch Intern Med. 1972;129(4):642-647. doi:10.1001/archinte.1972.00320040118016