Ectopic ACTH Syndrome

Symptom complex due to ACTH production by non-pituitary neoplasms.
Also Known As:
ACTH Syndrome, Ectopic; ACTH Syndromes, Ectopic; Ectopic ACTH Syndromes; Syndrome, Ectopic ACTH; Syndromes, Ectopic ACTH
Networked: 286 relevant articles (15 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cushing Syndrome
2. Neoplasms (Cancer)
3. Adrenocortical Carcinoma
4. Carcinoma (Carcinomatosis)
5. Addison Disease (Addison's Disease)


1. Hirata, Yukio: 7 articles (01/2015 - 12/2005)
2. Izumiyama, Hajime: 5 articles (01/2011 - 12/2005)
3. Young, William F: 4 articles (02/2015 - 04/2004)
4. Tani, Yuji: 4 articles (01/2015 - 03/2008)
5. Doi, Masaru: 4 articles (01/2015 - 12/2005)
6. Sakihara, Satoru: 4 articles (01/2014 - 01/2009)
7. Suda, Toshihiro: 4 articles (01/2014 - 01/2009)
8. Kageyama, Kazunori: 4 articles (01/2014 - 01/2009)
9. Bertagna, X: 4 articles (07/2008 - 04/2001)
10. Erickson, Dana: 3 articles (02/2015 - 04/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ectopic ACTH Syndrome:
1. Adrenocorticotropic Hormone (ACTH)FDA Link
2. Hydrocortisone (Cortisol)FDA LinkGeneric
3. Octreotide (Sandostatin)FDA LinkGeneric
4. Somatostatin (Somatotropin Release-Inhibiting Factor)IBA
5. Ketoconazole (Nizoral)FDA LinkGeneric
6. Dexamethasone (Maxidex)FDA LinkGeneric
7. Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric
8. Somatostatin Receptors (Somatostatin Receptor)IBA
04/01/1994 - "Somatostatin receptor scintigraphy and the ectopic ACTH syndrome--the solution or just another test?"
01/01/2002 - "Ectopic ACTH syndrome: discrepancy between somatostatin receptor status in vivo and ex vivo, and between immunostaining and gene transcription for POMC and CRH."
02/01/2012 - "This case report demonstrates the management of ectopic ACTH-syndrome by molecularly -targeted therapy with dopamine receptor -agonists as well as improved detection of the ectopic ACTH source by novel imaging modalities, such as [(68)Ga-DOTA]-TATE PET specifically targeting somatostatin receptor subtype-2 with high affinity."
10/01/2006 - "We concluded that: 1) measurement of late-night or bedtime salivary cortisol is a useful approach to screen for Cushing's syndrome; 2) measurement of suppressed plasma ACTH by immunometric assay is useful to differentiate ACTH-dependent and -independent Cushing's syndrome; 3) inferior petrosal sinus sampling for ACTH should be performed in patients with ACTH-dependent hypercortisolism in whom a pituitary magnetic resonance imaging is normal or equivocal (in the absence of a pituitary ACTH gradient, prolactin levels should be measured to confirm the integrity of venous sampling); 4) computed tomography of the chest and abdomen and somatostatin receptor scintigraphy should be performed in patients with the occult ectopic ACTH syndrome; and 5) patients with Cushing's disease should be referred to a neurosurgeon with extensive experience operating on corticotroph microadenomas. "
9. Dopamine Agonists (Dopamine Agonist)IBA
10. CortisoneIBA

Therapies and Procedures

1. Adrenalectomy
2. Intravenous Injections
3. Combination Drug Therapy (Combination Chemotherapy)
4. Surgical Instruments (Clip)
10/01/1994 - "In three tumors responsible for the ectopic ACTH syndrome, CLIP peptides were predominant in two and phosphorylated molecules represented between 22% and 50% of immunoreactive materials. "
01/01/1988 - "It is often altered in non-pituitary tumors responsible for the ectopic ACTH syndrome: abnormal peptides may be generated (CLIP h beta MSH5-22) which can be detected in blood. "
11/01/1980 - "Adrenocorticotrophin, lipotrophin and the related peptides alpha-MSH, CLIP, beta-endorphin and met-enkephalin have been measured, and characterized chromatographically in tumour extracts from seven patients with the ectopic ACTH syndrome. "
12/01/1989 - "In order to evaluate which of human (h) corticotrophin-like intermediary lobe peptide (CLIP) or h beta-melanocyte stimulating hormone5-22 (h beta MSH5-22) was the better marker of alternate pro-opiomelanocortin (POMC) processing, both peptides were simultaneously sought in the same tissue extracts from a normal human pituitary, six corticotrophic adenomas, and four non-pituitary tumours responsible for an ectopic ACTH syndrome. "
01/01/1981 - "ACTH, beta-LPH, gamma-LPH and beta-endorphin are observed in the plasma of normal subjects and patients with increased pituitary ACTH secretion, whereas cerebrospinal fluid contains ACTH, beta-LPH, gamma-LPH and beta-endorphin, a 31 000-molecular-weight putative precursor having ACTH, LPH and gamma-MSH immunoreactivities, as well as pro-gamma-MSH(1-77) and smaller immunoreactive gamma-MSH fragments, alpha-MSH was not observed in blood or cerebrospinal fluid but this pars intermedia peptide and corticotropin-like intermediate lobe peptide (CLIP) were both found in tumour tissues obtained from patients with the ectopic ACTH syndrome. "
5. Drug Therapy (Chemotherapy)