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Ectopic ACTH secretion syndrome

MedGen UID:
103
•Concept ID:
C0001231
•
Disease or Syndrome
Synonym: Ectopic ACTH syndrome
SNOMED CT: Hypercortisolism due to nonpituitary tumor (626004); Ectopic ACTH syndrome (626004); Ectopic adrenocorticotropic hormone secretion (237829004); Ectopic ACTH secretion (237829004); Ectopic ACTH secretion causing Cushing's syndrome (626004)
 
Monarch Initiative: MONDO:0043472

Definition

A syndrome characterized by abnormal secretion of adrenocorticotrophic hormone in conjunction with neoplastic growth occurring anywhere in the body. The most common associations are tumors of the bronchus (oat cell or carcinoid), thymic tumors (epithelial or carcinoid), and pancreatic endocrine tumor. (DeVita et al. Cancer, p 1364. 4th edition. Lippincott) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEctopic ACTH secretion syndrome

Professional guidelines

PubMed

Machine learning models for differential diagnosis of Cushing's disease and ectopic ACTH secretion syndrome.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2023 Jun;80(3):639-646. Epub 2023 Mar 18 doi: 10.1007/s12020-023-03341-7. PMID: 36933156
A noninvasive scoring model for the differential diagnosis of ACTH-dependent Cushing's syndrome: a retrospective analysis of 311 patients based on easy-to-use parameters.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2022 Oct;78(1):114-122. Epub 2022 Aug 4 doi: 10.1007/s12020-022-03081-0. PMID: 35925471
Management of Cushing's syndrome due to ectopic adrenocorticotropin secretion with 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane: findings in 23 patients from a single center.
Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugué MA, Coste J, Bertagna X, Bertherat J
J Clin Endocrinol Metab 2010 Feb;95(2):537-44. Epub 2010 Jan 8 doi: 10.1210/jc.2009-1317. PMID: 20061433

Recent clinical studies

Etiology

A noninvasive scoring model for the differential diagnosis of ACTH-dependent Cushing's syndrome: a retrospective analysis of 311 patients based on easy-to-use parameters.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2022 Oct;78(1):114-122. Epub 2022 Aug 4 doi: 10.1007/s12020-022-03081-0. PMID: 35925471
Median 10 years follow-up of patients with covert Cushing's syndrome: a case series.
Najafipour F, Bahrami A, Niafar M, Houshyar J, Halimi M, Sadra V
J Med Case Rep 2021 Oct 11;15(1):514. doi: 10.1186/s13256-021-03046-3. PMID: 34635153Free PMC Article
VENOUS SAMPLING FOR CUSHING DISEASE: COMPARISON OF INTERNAL JUGULAR VEIN AND INFERIOR PETROSAL SINUS SAMPLING.
Radvany MG, Quinones-Hinojosa A, Gallia GL, Wand GS, Salvatori R
Endocr Pract 2016 Sep;22(9):1057-61. Epub 2016 May 23 doi: 10.4158/EP161266.OR. PMID: 27214296
Management of Cushing's syndrome due to ectopic adrenocorticotropin secretion with 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane: findings in 23 patients from a single center.
Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugué MA, Coste J, Bertagna X, Bertherat J
J Clin Endocrinol Metab 2010 Feb;95(2):537-44. Epub 2010 Jan 8 doi: 10.1210/jc.2009-1317. PMID: 20061433

Diagnosis

Machine learning models for differential diagnosis of Cushing's disease and ectopic ACTH secretion syndrome.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2023 Jun;80(3):639-646. Epub 2023 Mar 18 doi: 10.1007/s12020-023-03341-7. PMID: 36933156
A noninvasive scoring model for the differential diagnosis of ACTH-dependent Cushing's syndrome: a retrospective analysis of 311 patients based on easy-to-use parameters.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2022 Oct;78(1):114-122. Epub 2022 Aug 4 doi: 10.1007/s12020-022-03081-0. PMID: 35925471
Median 10 years follow-up of patients with covert Cushing's syndrome: a case series.
Najafipour F, Bahrami A, Niafar M, Houshyar J, Halimi M, Sadra V
J Med Case Rep 2021 Oct 11;15(1):514. doi: 10.1186/s13256-021-03046-3. PMID: 34635153Free PMC Article
VENOUS SAMPLING FOR CUSHING DISEASE: COMPARISON OF INTERNAL JUGULAR VEIN AND INFERIOR PETROSAL SINUS SAMPLING.
Radvany MG, Quinones-Hinojosa A, Gallia GL, Wand GS, Salvatori R
Endocr Pract 2016 Sep;22(9):1057-61. Epub 2016 May 23 doi: 10.4158/EP161266.OR. PMID: 27214296
Ectopic ACTH-secreting syndrome: a single center experience report with a high prevalence of occult tumor.
Hernández I, Espinosa-de-los-Monteros AL, Mendoza V, Cheng S, Molina M, Sosa E, Mercado M
Arch Med Res 2006 Nov;37(8):976-80. doi: 10.1016/j.arcmed.2006.05.015. PMID: 17045113

Therapy

Machine learning models for differential diagnosis of Cushing's disease and ectopic ACTH secretion syndrome.
Lyu X, Zhang D, Pan H, Zhu H, Chen S, Lu L
Endocrine 2023 Jun;80(3):639-646. Epub 2023 Mar 18 doi: 10.1007/s12020-023-03341-7. PMID: 36933156
Management of Cushing's syndrome due to ectopic adrenocorticotropin secretion with 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane: findings in 23 patients from a single center.
Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugué MA, Coste J, Bertagna X, Bertherat J
J Clin Endocrinol Metab 2010 Feb;95(2):537-44. Epub 2010 Jan 8 doi: 10.1210/jc.2009-1317. PMID: 20061433

Prognosis

Median 10 years follow-up of patients with covert Cushing's syndrome: a case series.
Najafipour F, Bahrami A, Niafar M, Houshyar J, Halimi M, Sadra V
J Med Case Rep 2021 Oct 11;15(1):514. doi: 10.1186/s13256-021-03046-3. PMID: 34635153Free PMC Article
VENOUS SAMPLING FOR CUSHING DISEASE: COMPARISON OF INTERNAL JUGULAR VEIN AND INFERIOR PETROSAL SINUS SAMPLING.
Radvany MG, Quinones-Hinojosa A, Gallia GL, Wand GS, Salvatori R
Endocr Pract 2016 Sep;22(9):1057-61. Epub 2016 May 23 doi: 10.4158/EP161266.OR. PMID: 27214296

Clinical prediction guides

VENOUS SAMPLING FOR CUSHING DISEASE: COMPARISON OF INTERNAL JUGULAR VEIN AND INFERIOR PETROSAL SINUS SAMPLING.
Radvany MG, Quinones-Hinojosa A, Gallia GL, Wand GS, Salvatori R
Endocr Pract 2016 Sep;22(9):1057-61. Epub 2016 May 23 doi: 10.4158/EP161266.OR. PMID: 27214296
Management of Cushing's syndrome due to ectopic adrenocorticotropin secretion with 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane: findings in 23 patients from a single center.
Donadille B, Groussin L, Waintrop C, Abbas H, Tenenbaum F, Dugué MA, Coste J, Bertagna X, Bertherat J
J Clin Endocrinol Metab 2010 Feb;95(2):537-44. Epub 2010 Jan 8 doi: 10.1210/jc.2009-1317. PMID: 20061433

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