| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
Endocrine Research Laboratory, Departments of Medicine and Radiology, St. Luke’s Medical Center Milwaukee, Wisconsin 53215
Address all correspondence and requests for reprints to: James W. Findling, M.D., Department of Medicine, St. Luke’s Medical Center, 2901 West Kinnickinnic River Parkway, Suite 503, Milwaukee, Wisconsin 53215.
The clinical, biochemical, and radiographic features of ectopic ACTH-dependent Cushing’s syndrome are often indistinguishable from those of pituitary ACTH-dependent hypercortisolism (Cushing’s disease). We prospectively evaluated 29 patients with ACTH-dependent hypercortisolism by means of bilateral inferior petrosal sinus ACTH sampling with ovine CRH (oCRH) stimulation. Patients with Cushing’s disease (n = 20) had a maximal basal inferior petrosal sinus to peripheral ACTH ratio (IPS:P-ACTH) of 11.7 ± 4.4 (±SE) from the dominant IPS, which increased to 50.8 ± 18.3 after oCRH administration. Bilateral IPS sampling was necessary to correctly identify patients with Cushing’s disease, since the maximal basal nondominant IPS:P-ACTH was less than 2.0 in over 50% of the patients and remained less than 2.0 after oCRH administration in one third. In contrast, patients with occult ectopic ACTHsecreting neoplasms (n = 9) had maximal basal IPS:P-ACTH of 1.2 ± 0.1 that did not change after oCRH administration. Occult ectopic ACTH-secreting neoplasms were found in 7 of 9 patients from 0.4–14 yr after the recognition of Cushing’s syndrome, and 4 of these patients had intermittent hypercortisolism with prolonged periods of remission. Selective endobronchial lavage for ACTH correctly localized a radiologically occult ACTH-secreting bronchial carcinoid in 1 patient, and magnetic resonance imaging identified a similar neoplasm in a patient with a normal chest computed tomographic scan.
Basal ACTH and urinary free cortisol excretion were significantly higher in patients with ectopic ACTH than in those with Cushing’s disease, but overlap existed between groups. High dose dexamethasone suppression testing inaccurately classified 24% of patients, and radiological imaging of the pituitary and adrenal glands was misleading. The occult ectopic ACTH syndrome is a common form of ACTH-dependent hypercortisolism that cannot be distinguished from Cushing’s disease with routine clinical studies. The accurate differential diagnosis of ACTHdependent Cushing’s syndrome requires bilateral inferior petrosal sinus ACTH sampling with oCRH stimulation.
Received December 5, 1990.
This article has been cited by other articles:
 |
|
 |
|
  P. Burman, A. Lethagen, K. Ivancev, L. Johansson, and A. Sundin Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling Eur. J. Endocrinol., October 1, 2008; 159(4): 483 - 488. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Hayashi, M. Kurimoto, M. Kubo, N. Kuwayama, K. Kurosaki, S. Nagai, and S. Endo The Impact of Cavernous Sinus Drainage Pattern on the Results of Venous Sampling in Patients With Suspected Cushing Syndrome AJNR Am. J. Neuroradiol., January 1, 2008; 29(1): 69 - 72. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Kidambi, H. Raff, and J. W Findling Limitations of nocturnal salivary cortisol and urine free cortisol in the diagnosis of mild Cushing's syndrome Eur. J. Endocrinol., December 1, 2007; 157(6): 725 - 731. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F Castinetti, I Morange, H Dufour, P Jaquet, B Conte-Devolx, N Girard, and T Brue Desmopressin test during petrosal sinus sampling: a valuable tool to discriminate pituitary or ectopic ACTH-dependent Cushing's syndrome Eur. J. Endocrinol., September 1, 2007; 157(3): 271 - 277. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. R. Salgado, M. C. B V. Fragoso, M. Knoepfelmacher, M. C. Machado, S. Domenice, M. A. A. Pereira, and B. B. de Mendonca Ectopic ACTH syndrome: our experience with 25 cases. Eur. J. Endocrinol., November 1, 2006; 155(5): 725 - 733. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. W. Findling and H. Raff Cushing's Syndrome: Important Issues in Diagnosis and Management J. Clin. Endocrinol. Metab., October 1, 2006; 91(10): 3746 - 3753. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Ilias, D. J. Torpy, K. Pacak, N. Mullen, R. A. Wesley, and L. K. Nieman Cushing's Syndrome Due to Ectopic Corticotropin Secretion: Twenty Years' Experience at the National Institutes of Health J. Clin. Endocrinol. Metab., August 1, 2005; 90(8): 4955 - 4962. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. W. Findling, M. E. Kehoe, and H. Raff Identification of Patients with Cushing's Disease with Negative Pituitary Adrenocorticotropin Gradients during Inferior Petrosal Sinus Sampling: Prolactin as an Index of Pituitary Venous Effluent J. Clin. Endocrinol. Metab., December 1, 2004; 89(12): 6005 - 6009. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Swearingen, L. Katznelson, K. Miller, S. Grinspoon, A. Waltman, D. J. Dorer, A. Klibanski, and B. M. K. Biller Diagnostic Errors after Inferior Petrosal Sinus Sampling J. Clin. Endocrinol. Metab., August 1, 2004; 89(8): 3752 - 3763. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Ilias, R. Chang, K. Pacak, E. H. Oldfield, R. Wesley, J. Doppman, and L. K. Nieman Jugular Venous Sampling: An Alternative to Petrosal Sinus Sampling for the Diagnostic Evaluation of Adrenocorticotropic Hormone-Dependent Cushing's Syndrome J. Clin. Endocrinol. Metab., August 1, 2004; 89(8): 3795 - 3800. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. M. Isidori, G. A. Kaltsas, S. Mohammed, D. G. Morris, P. Jenkins, S. L. Chew, J. P. Monson, G. M. Besser, and A. B. Grossman Discriminatory Value of the Low-Dose Dexamethasone Suppression Test in Establishing the Diagnosis and Differential Diagnosis of Cushing's Syndrome J. Clin. Endocrinol. Metab., November 1, 2003; 88(11): 5299 - 5306. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Raff and J. W. Findling A Physiologic Approach to Diagnosis of the Cushing Syndrome Ann Intern Med, June 17, 2003; 138(12): 980 - 991. [Full Text] [PDF]
|
|
|
|
|
|
P. Loli, F. Vignati, E. Grossrubatscher, P. Dalino, M. Possa, F. Zurleni, G. Lomuscio, O. Rossetti, M. Ravini, A. Vanzulli, et al. Management of Occult Adrenocorticotropin-Secreting Bronchial Carcinoids: Limits of Endocrine Testing and Imaging Techniques J. Clin. Endocrinol. Metab., March 1, 2003; 88(3): 1029 - 1035. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Boscaro, L. Barzon, and N. Sonino The Diagnosis of Cushing's Syndrome: Atypical Presentations and Laboratory Shortcomings Arch Intern Med, November 13, 2000; 160(20): 3045 - 3053. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. S. Bonelli, J. Huston III, P. C. Carpenter, D. Erickson, W. F. Young Jr., and F. B. Meyer Adrenocorticotropic hormone-dependent Cushing's Syndrome: Sensitivity and Specificity of Inferior Petrosal Sinus Sampling AJNR Am. J. Neuroradiol., April 1, 2000; 21(4): 690 - 696. [Abstract] [Full Text]
|
|
|
|
|
|
M. I. Wiggam, A. P. Heaney, E. M. McIlrath, D. R. McCance, B. Sheridan, D. R. Hadden, and A. B. Atkinson Bilateral Inferior Petrosal Sinus Sampling in the Differential Diagnosis of Adrenocorticotropin-Dependent Cushing's Syndrome: A Comparison with Other Diagnostic Tests J. Clin. Endocrinol. Metab., April 1, 2000; 85(4): 1525 - 1532. [Abstract] [Full Text]
|
|
|
|
 |
|
 G. G. Klee Maximizing Efficacy of Endocrine Tests: Importance of Decision-focused Testing Strategies and Appropriate Patient Preparation Clin. Chem., August 1, 1999; 45(8): 1323 - 1330. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Tabarin, N. Valli, P. Chanson, Y. Bachelot, V. Rohmer, V. Bex-Bachellerie, B. Catargi, P. Roger, and F. Laurent Usefulness of Somatostatin Receptor Scintigraphy in Patients with Occult Ectopic Adrenocorticotropin Syndrome J. Clin. Endocrinol. Metab., April 1, 1999; 84(4): 1193 - 1202. [Abstract] [Full Text]
|
|
|
|
|
|
G. A. Kaltsas, M. G. Giannulis, J. D. C. Newell-Price, J. E. Dacie, C. Thakkar, F. Afshar, J. P. Monson, A. B. Grossman, G. M. Besser, and P. J. Trainer A Critical Analysis of the Value of Simultaneous Inferior Petrosal Sinus Sampling in Cushing's Disease and the Occult Ectopic Adrenocorticotropin Syndrome J. Clin. Endocrinol. Metab., February 1, 1999; 84(2): 487 - 492. [Abstract] [Full Text]
|
|
|
|
|
|
J. L. Doppman, R. Chang, E. H. Oldfield, G. Chrousos, C. A. Stratakis, and L. K. Nieman The Hypoplastic Inferior Petrosal Sinus: A Potential Source of False-Negative Results in Petrosal Sampling for Cushing's Disease J. Clin. Endocrinol. Metab., February 1, 1999; 84(2): 533 - 540. [Abstract] [Full Text]
|
|
|
|
 |
|
 J. Newell-Price, P. Trainer, M. Besser, and A. Grossman The Diagnosis and Differential Diagnosis of Cushing's Syndrome and Pseudo-Cushing's States Endocr. Rev., October 1, 1998; 19(5): 647 - 672. [Abstract] [Full Text]
|
|
|
|
|
|
H. Raff, J. L. Raff, and J. W. Findling Late-Night Salivary Cortisol as a Screening Test for Cushing's Syndrome J. Clin. Endocrinol. Metab., August 1, 1998; 83(8): 2681 - 2686. [Abstract] [Full Text]
|
|
|
|
|
|
D. C. Aron, H. Raff, and J. W. Findling Effectiveness Versus Efficacy: The Limited Value in Clinical Practice of High Dose Dexamethasone Suppression Testing in the Differential Diagnosis of Adrenocorticotropin-Dependent Cushing's Syndrome J. Clin. Endocrinol. Metab., June 1, 1997; 82(6): 1780 - 1785. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. N. Orth Cushing's Syndrome N. Engl. J. Med., March 23, 1995; 332(12): 791 - 803. [Full Text] [PDF]
|
|
|
|
|
|
R. Kreisberg Half a Loaf N. Engl. J. Med., May 5, 1994; 330(18): 1295 - 1299. [Full Text]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Endocrinology | Endocrine Reviews | J. Clin. End. & Metab. |
| Molecular Endocrinology | Recent Prog. Horm. Res. | All Endocrine Journals |
