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ACTH-independent Cushing syndrome

MedGen UID:
978155
Concept ID:
CN294278
Disease or Syndrome
Synonyms: Adrenal Cushing syndrome; adrenal Cushing syndrome; Adrenocorticotropic hormone-independent Cushing syndrome; adrenocorticotropic hormone-independent Cushing syndrome; Corticotropin-independent Cushing syndrome; corticotropin-independent Cushing syndrome
 
Related genes: ARMC5, GNAS
 
Monarch Initiative: MONDO:0020529
Orphanet: ORPHA99893

Definition

A form of endogenous Cushing syndrome (CS) that may result from excess secretion of cortisol by either a unilateral and benign (adrenocortical adenoma: 55-60%) or malignant (adrenocortical carcinoma: 35-40 %) adrenocortical tumor or by bilateral adrenal secretion by macronodular adrenal hyperplasia (AIMAH), as an isolated disease or as part of McCune-Albright syndrome (MAS), or by primary pigmented nodular adrenocortical disease (PPNAD), as an isolated disease or as part of Carney complex (CNC). [from ORDO]

Professional guidelines

PubMed

Avgerinos PC, Yanovski JA, Oldfield EH, Nieman LK, Cutler GB Jr
Ann Intern Med 1994 Sep 1;121(5):318-27. doi: 10.7326/0003-4819-121-5-199409010-00002. PMID: 8042820

Recent clinical studies

Etiology

De Leo A, Mosconi C, Zavatta G, Tucci L, Nanni C, Selva S, Balacchi C, Ceccarelli C, Santini D, Pantaleo MA, Minni F, Fanti S, Golfieri R, Pagotto U, Vicennati V, Di Dalmazi G
J Endocrinol Invest 2020 Sep;43(9):1197-1204. Epub 2020 Feb 15 doi: 10.1007/s40618-020-01198-5. PMID: 32062826
London E, Lodish M, Keil M, Lyssikatos C, de la Luz Sierra M, Nesterova M, Stratakis CA
Horm Metab Res 2014 Nov;46(12):897-903. Epub 2014 Oct 8 doi: 10.1055/s-0034-1389956. PMID: 25295416
Aggarwal S, Yadav K, Sharma AP, Sethi V
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):324-8. doi: 10.1097/SLE.0b013e318290126d. PMID: 23752002
Xu Y, Rui W, Qi Y, Zhang C, Zhao J, Wang X, Wu Y, Zhu Q, Shen Z, Ning G, Zhu Y
World J Surg 2013 Jul;37(7):1626-32. doi: 10.1007/s00268-013-2059-9. PMID: 23592061
Rockall AG, Babar SA, Sohaib SA, Isidori AM, Diaz-Cano S, Monson JP, Grossman AB, Reznek RH
Radiographics 2004 Mar-Apr;24(2):435-52. doi: 10.1148/rg.242035092. PMID: 15026592

Diagnosis

Zhong S, Zhang T, He M, Yu H, Liu Z, Li Z, Song X, Xu X
Front Endocrinol (Lausanne) 2022;13:797021. Epub 2022 Feb 9 doi: 10.3389/fendo.2022.797021. PMID: 35222268Free PMC Article
Aouinati Y, Mjabber A, Haraj NE, Aziz SE, Chadli A
Pan Afr Med J 2021;38:367. Epub 2021 Apr 15 doi: 10.11604/pamj.2021.38.367.26040. PMID: 34367446Free PMC Article
Chen LS, Singh RJ
Crit Rev Clin Lab Sci 2018 Mar;55(2):115-128. Epub 2018 Jan 22 doi: 10.1080/10408363.2018.1425975. PMID: 29357735
Xu Y, Rui W, Qi Y, Zhang C, Zhao J, Wang X, Wu Y, Zhu Q, Shen Z, Ning G, Zhu Y
World J Surg 2013 Jul;37(7):1626-32. doi: 10.1007/s00268-013-2059-9. PMID: 23592061
Rockall AG, Babar SA, Sohaib SA, Isidori AM, Diaz-Cano S, Monson JP, Grossman AB, Reznek RH
Radiographics 2004 Mar-Apr;24(2):435-52. doi: 10.1148/rg.242035092. PMID: 15026592

Therapy

London E, Lodish M, Keil M, Lyssikatos C, de la Luz Sierra M, Nesterova M, Stratakis CA
Horm Metab Res 2014 Nov;46(12):897-903. Epub 2014 Oct 8 doi: 10.1055/s-0034-1389956. PMID: 25295416

Prognosis

De Leo A, Mosconi C, Zavatta G, Tucci L, Nanni C, Selva S, Balacchi C, Ceccarelli C, Santini D, Pantaleo MA, Minni F, Fanti S, Golfieri R, Pagotto U, Vicennati V, Di Dalmazi G
J Endocrinol Invest 2020 Sep;43(9):1197-1204. Epub 2020 Feb 15 doi: 10.1007/s40618-020-01198-5. PMID: 32062826
Hurtado MD, Cortes T, Natt N, Young WF Jr, Bancos I
Clin Endocrinol (Oxf) 2018 Dec;89(6):721-733. Epub 2018 Jul 23 doi: 10.1111/cen.13803. PMID: 29968420Free PMC Article
Chen LS, Singh RJ
Crit Rev Clin Lab Sci 2018 Mar;55(2):115-128. Epub 2018 Jan 22 doi: 10.1080/10408363.2018.1425975. PMID: 29357735
Aggarwal S, Yadav K, Sharma AP, Sethi V
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):324-8. doi: 10.1097/SLE.0b013e318290126d. PMID: 23752002
Xu Y, Rui W, Qi Y, Zhang C, Zhao J, Wang X, Wu Y, Zhu Q, Shen Z, Ning G, Zhu Y
World J Surg 2013 Jul;37(7):1626-32. doi: 10.1007/s00268-013-2059-9. PMID: 23592061

Clinical prediction guides

Sol B, Carprieaux M, De Leu N
BMJ Case Rep 2023 Jul 6;16(7) doi: 10.1136/bcr-2022-254099. PMID: 37419498Free PMC Article
De Leo A, Mosconi C, Zavatta G, Tucci L, Nanni C, Selva S, Balacchi C, Ceccarelli C, Santini D, Pantaleo MA, Minni F, Fanti S, Golfieri R, Pagotto U, Vicennati V, Di Dalmazi G
J Endocrinol Invest 2020 Sep;43(9):1197-1204. Epub 2020 Feb 15 doi: 10.1007/s40618-020-01198-5. PMID: 32062826
Hurtado MD, Cortes T, Natt N, Young WF Jr, Bancos I
Clin Endocrinol (Oxf) 2018 Dec;89(6):721-733. Epub 2018 Jul 23 doi: 10.1111/cen.13803. PMID: 29968420Free PMC Article
Chen LS, Singh RJ
Crit Rev Clin Lab Sci 2018 Mar;55(2):115-128. Epub 2018 Jan 22 doi: 10.1080/10408363.2018.1425975. PMID: 29357735
Bernichtein S, Alevizaki M, Huhtaniemi I
Trends Endocrinol Metab 2008 Sep;19(7):231-8. Epub 2008 Aug 6 doi: 10.1016/j.tem.2008.06.003. PMID: 18691899

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