Cushing's syndrome due to ectopic corticotropin secretion: twenty years' experience at the National Institutes of Health.
BACKGROUND
Ectopic ACTH secretion (EAS) is difficult to diagnose and treat. We present our experience with EAS from 1983 to 2004.


METHODS
The study was performed at a tertiary care clinical research center.


METHODS
Ninety patients, aged 8-72 yr, including 48 females were included in the study.


METHODS
Tests included 8 mg dexamethasone suppression, CRH stimulation, inferior petrosal sinus sampling (IPSS), computed tomography, octreotide scan, magnetic resonance imaging, and/or venous sampling. Therapies, pathological examinations, and survival were noted.


RESULTS
Eighty-six to 94% of patients did not respond to CRH or dexamethasone suppression, whereas 66 of 67 had negative IPSS. To control hypercortisolism, 62 patients received medical treatment, and 33 had bilateral adrenalectomy. Imaging localized tumors in 67 of 90 patients. Surgery confirmed an ACTH-secreting tumor in 59 of 66 patients and cured 65%. Nonthymic carcinoids took longest to localize. Deaths included three of 35 with pulmonary carcinoid, two of five with thymic carcinoid, four of six with gastrinoma, two of 13 with neuroendocrine tumor, two of two with medullary thyroid cancer, one of five with pheochromocytoma, three of three with small-cell lung cancer, and two of 17 with occult tumor. Patients with other carcinoids and ethesioneuroblastoma are alive.


CONCLUSIONS
IPSS best identifies EAS. Initial failed localization is common and suggests pulmonary carcinoid. Although only 47% achieved cure, survival is good except in patients with small-cell lung cancer, medullary thyroid cancer, and gastrinoma.
Ilias
Ioannis
I
Pediatric and Reproductive Endocrinology Branch, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892-1109, USA.
Torpy
David J
DJ
Pacak
Karel
K
Mullen
Nancy
N
Wesley
Robert A
RA
Nieman
Lynnette K
LK
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D000368:Aged; D002276:Carcinoid Tumor / Q000150:complications* / Q000378:metabolism / Q000401:mortality; D002648:Child; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000378:metabolism / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009316:National Institutes of Health (U.S.); D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D014481:United States
D000324:Adrenocorticotropic Hormone