Corticotropin-dependent Cushing's syndrome in older people: presentation of five cases and therapeutical use of ketoconazole.
BACKGROUND
Cushing's syndrome is a rare disorder. The corticotropin (ACTH)-dependent form of this syndrome generally results either from excessive ACTH secretion by a pituitary adenoma or ectopic secretion by a malignant tumor. Theoretically, the latter type can be assumed to occur more frequently in old age as the incidence of malignancy increases.


METHODS
Diagnostic procedures for these five cases of Cushing's syndrome consisted of 24-hour urinary cortisol excretion, plasma ACTH and serum cortisol levels, oCRH stimulatory test, low-dose and high-dose dexamethasone suppression tests, CT scan or MR imaging of the pituitary region, and bilateral inferior petrosal sinus sampling. Patients were treated with ketoconazole, if possible, and evaluated according to clinical response and 24-hour urinary cortisol excretion.


METHODS
The five cases presented were selected on the basis of age--75 years or older--from a total of about 100 patients presenting with Cushing's syndrome. In only three cases were signs of hypercorticism found on clinical examination. The other two patients were evaluated for adrenocortical excess because of severe hypokalemia and the fortuitous finding of enlarged adrenal glands on CT scan, respectively.


RESULTS
As a result of endocrine testing, pituitary-dependent Cushing's disease was suspected in three patients and ectopic Cushing's syndrome in two patients. Imaging techniques demonstrated only one pituitary adenoma in the first three patients and a lung tumor in one of the latter two patients. Inferior petrosal sinus sampling confirmed the suspected origin of the Cushing's syndrome in the three patients in which this procedure was performed. All three patients with pituitary-dependent Cushing's disease underwent successful clinical and biochemical treatment with ketoconazole.


CONCLUSIONS
Pituitary-dependent Cushing's disease may occur more frequently in patients older than 75 years of age than has previously been assumed. Because surgical treatment is not always easily tolerated by older patients, the steroidogenesis inhibitor, ketoconazole, can be a valuable alternative for the control of hypercorticism.
Berwaerts
J J
JJ
Department of Endocrinology, Middelheim Hospital, Antwerp, Belgium.
Verhelst
J A
JA
Verhaert
G C
GC
Verhaegen
A A
AA
Abs
R E
RE
D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000368:Aged; D000369:Aged, 80 and over; D003480:Cushing Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007654:Ketoconazole / Q000627:therapeutic use*; D008297:Male; D010902:Pituitary Gland / Q000473:pathology
D000324:Adrenocorticotropic Hormone; D007654:Ketoconazole; D006854:Hydrocortisone