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Ectopic Cushing syndrome

MedGen UID:
797686
•Concept ID:
CN207427
•
Disease or Syndrome
Synonyms: Adrenocorticotropic hormone secretion syndrome; adrenocorticotropic hormone secretion syndrome; Cushing syndrome due to ectopic ACTH secretion; ectopic ACTH secreting tumor; Ectopic ACTH secreting tumor; ectopic ACTH secreting tumour; ectopic Cushing syndrome; occult ectopic ACTH secretion; Occult ectopic ACTH secretion; Paraneoplastic Cushing syndrome; paraneoplastic Cushing syndrome
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0020527
Orphanet: ORPHA99889

Definition

Cushing syndrome due to ectopic (adrenocorticotropic hormone) ACTH secretion (EAS) is a form of ACTH-dependent Cushing syndrome (see this term) caused by excess secretion of ACTH by a benign or, more often, malignant non-pituitary tumor. [from ORDO]

Professional guidelines

PubMed

Treatment of Cushing's syndrome with osilodrostat: practical applications of recent studies with case examples.
Fleseriu M, Biller BMK
Pituitary 2022 Dec;25(6):795-809. Epub 2022 Aug 24 doi: 10.1007/s11102-022-01268-2. PMID: 36002784Free PMC Article
Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
Ito T, Jensen RT
Expert Opin Pharmacother 2021 Apr;22(6):685-693. Epub 2020 Nov 11 doi: 10.1080/14656566.2020.1845651. PMID: 33131345Free PMC Article
MANAGEMENT OF ENDOCRINE DISEASE: Cushing's syndrome due to ectopic ACTH secretion: an expert operational opinion.
Young J, Haissaguerre M, Viera-Pinto O, Chabre O, Baudin E, Tabarin A
Eur J Endocrinol 2020 Apr;182(4):R29-R58. doi: 10.1530/EJE-19-0877. PMID: 31999619

Recent clinical studies

Etiology

Paediatric Cushing syndrome: a prospective, multisite, observational cohort study.
Tatsi C, Kamilaris C, Keil M, Saidkhodjaeva L, Faucz FR, Chittiboina P, Stratakis CA
Lancet Child Adolesc Health 2024 Jan;8(1):51-62. doi: 10.1016/S2352-4642(23)00264-X. PMID: 38097317Free PMC Article
Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
Landry JP, Clemente-Gutierrez U, Pieterman CRC, Chiang YJ, Waguespack SG, Jimenez C, Habra MA, Halperin DM, Fisher SB, Graham PH, Perrier ND
Surgery 2022 Aug;172(2):559-566. Epub 2022 Apr 16 doi: 10.1016/j.surg.2022.03.014. PMID: 35437162Free PMC Article
Ectopic Cushing syndrome in Colombia.
Lopez-Montoya V, Gutierrez-Restrepo J, Grajales JLT, Aristizabal N, Pantoja D, Roman-Gonzalez A, Jimenez C
Arch Endocrinol Metab 2021 May 18;64(6):687-694. doi: 10.20945/2359-3997000000271. PMID: 34033277Free PMC Article
Two types of ectopic Cushing syndrome or a continuum? Review.
Araujo Castro M, Marazuela Azpiroz M
Pituitary 2018 Oct;21(5):535-544. doi: 10.1007/s11102-018-0894-2. PMID: 29766359
Ectopic Cushing syndrome: Report of 9 cases.
Araujo Castro M, Palacios García N, Aller Pardo J, Izquierdo Alvarez C, Armengod Grao L, Estrada García J
Endocrinol Diabetes Nutr (Engl Ed) 2018 May;65(5):255-264. Epub 2018 Apr 11 doi: 10.1016/j.endinu.2018.02.001. PMID: 29655957

Diagnosis

Somatostatin receptor PET beyond the neuroendocrine tumors of the gastrointestinal tract - the review of literature.
Zogala D
Klin Onkol 2021 Summer;34(4):291-299. doi: 10.48095/ccko2021291. PMID: 34905930
Ectopic Cushing syndrome in Colombia.
Lopez-Montoya V, Gutierrez-Restrepo J, Grajales JLT, Aristizabal N, Pantoja D, Roman-Gonzalez A, Jimenez C
Arch Endocrinol Metab 2021 May 18;64(6):687-694. doi: 10.20945/2359-3997000000271. PMID: 34033277Free PMC Article
Functional imaging in ectopic Cushing syndrome.
Grigoryan S, Avram AM, Turcu AF
Curr Opin Endocrinol Diabetes Obes 2020 Jun;27(3):146-154. doi: 10.1097/MED.0000000000000541. PMID: 32250975Free PMC Article
Ectopic Corticotropin-Producing Neuroendocrine Tumor of the Pancreas Treated With 177Lu DOTATATE Induction and Maintenance Peptide Receptor Radionuclide Therapy.
Makis W, McCann K, Riauka TA, McEwan AJ
Clin Nucl Med 2016 Jan;41(1):50-2. doi: 10.1097/RLU.0000000000000971. PMID: 26359569
Multiple pulmonary atypical carcinoids presenting with long-standing Cushing syndrome masked by pulmonary tuberculosis.
Jang BG, Kim SY, Park SH
Pathol Int 2009 Jun;59(6):399-404. doi: 10.1111/j.1440-1827.2009.02384.x. PMID: 19490471

Therapy

Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
Landry JP, Clemente-Gutierrez U, Pieterman CRC, Chiang YJ, Waguespack SG, Jimenez C, Habra MA, Halperin DM, Fisher SB, Graham PH, Perrier ND
Surgery 2022 Aug;172(2):559-566. Epub 2022 Apr 16 doi: 10.1016/j.surg.2022.03.014. PMID: 35437162Free PMC Article
Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre.
Paleń-Tytko JE, Przybylik-Mazurek EM, Rzepka EJ, Pach DM, Sowa-Staszczak AS, Gilis-Januszewska A, Hubalewska-Dydejczyk AB
PLoS One 2020;15(11):e0242679. Epub 2020 Nov 25 doi: 10.1371/journal.pone.0242679. PMID: 33237923Free PMC Article
Ectopic Cushing syndrome: Report of 9 cases.
Araujo Castro M, Palacios García N, Aller Pardo J, Izquierdo Alvarez C, Armengod Grao L, Estrada García J
Endocrinol Diabetes Nutr (Engl Ed) 2018 May;65(5):255-264. Epub 2018 Apr 11 doi: 10.1016/j.endinu.2018.02.001. PMID: 29655957
Multiple pulmonary atypical carcinoids presenting with long-standing Cushing syndrome masked by pulmonary tuberculosis.
Jang BG, Kim SY, Park SH
Pathol Int 2009 Jun;59(6):399-404. doi: 10.1111/j.1440-1827.2009.02384.x. PMID: 19490471
Multidisciplinary approach including receptor radionuclide therapy with 90Y-DOTATOC ([90Y-DOTA0, Tyr3]-octreotide) and 177Lu-DOTATATE ([177Lu-DOTA0, Tyr3]-octreotate) in ectopic cushing syndrome from a metastatic gastrinoma: a promising proposal.
Davì MV, Bodei L, Ferdeghini M, Falconi M, Testoni M, Paganelli G, Oliani C, Lo Cascio V, Francia G
Endocr Pract 2008 Mar;14(2):213-8. doi: 10.4158/EP.14.2.213. PMID: 18308661

Prognosis

Paediatric Cushing syndrome: a prospective, multisite, observational cohort study.
Tatsi C, Kamilaris C, Keil M, Saidkhodjaeva L, Faucz FR, Chittiboina P, Stratakis CA
Lancet Child Adolesc Health 2024 Jan;8(1):51-62. doi: 10.1016/S2352-4642(23)00264-X. PMID: 38097317Free PMC Article
Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
Landry JP, Clemente-Gutierrez U, Pieterman CRC, Chiang YJ, Waguespack SG, Jimenez C, Habra MA, Halperin DM, Fisher SB, Graham PH, Perrier ND
Surgery 2022 Aug;172(2):559-566. Epub 2022 Apr 16 doi: 10.1016/j.surg.2022.03.014. PMID: 35437162Free PMC Article
Two types of ectopic Cushing syndrome or a continuum? Review.
Araujo Castro M, Marazuela Azpiroz M
Pituitary 2018 Oct;21(5):535-544. doi: 10.1007/s11102-018-0894-2. PMID: 29766359
Ectopic Cushing syndrome: Report of 9 cases.
Araujo Castro M, Palacios García N, Aller Pardo J, Izquierdo Alvarez C, Armengod Grao L, Estrada García J
Endocrinol Diabetes Nutr (Engl Ed) 2018 May;65(5):255-264. Epub 2018 Apr 11 doi: 10.1016/j.endinu.2018.02.001. PMID: 29655957
Characterizing an ectopic secreting carcinoid with indium-111-DTPA-D-Phe-pentetreotide.
Briganti V, Mannelli M, La Cava G, Peri A, Meldolesi U, Masi R, Pupi A
J Nucl Med 1997 May;38(5):711-4. PMID: 9170433

Clinical prediction guides

Paediatric Cushing syndrome: a prospective, multisite, observational cohort study.
Tatsi C, Kamilaris C, Keil M, Saidkhodjaeva L, Faucz FR, Chittiboina P, Stratakis CA
Lancet Child Adolesc Health 2024 Jan;8(1):51-62. doi: 10.1016/S2352-4642(23)00264-X. PMID: 38097317Free PMC Article
Limited Invasive Protocol: Optimizing Diagnostic Modalities in Corticotropin Mediated Cushing Syndrome.
Gupta R, Walia R, Ahuja C, Dutta A, Bhadada SK, Bhansali A, Chhabra R, Singh A, Dhandapani S
Endocr Pract 2022 Aug;28(8):767-773. Epub 2022 May 4 doi: 10.1016/j.eprac.2022.04.013. PMID: 35525501
Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
Landry JP, Clemente-Gutierrez U, Pieterman CRC, Chiang YJ, Waguespack SG, Jimenez C, Habra MA, Halperin DM, Fisher SB, Graham PH, Perrier ND
Surgery 2022 Aug;172(2):559-566. Epub 2022 Apr 16 doi: 10.1016/j.surg.2022.03.014. PMID: 35437162Free PMC Article
Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre.
Paleń-Tytko JE, Przybylik-Mazurek EM, Rzepka EJ, Pach DM, Sowa-Staszczak AS, Gilis-Januszewska A, Hubalewska-Dydejczyk AB
PLoS One 2020;15(11):e0242679. Epub 2020 Nov 25 doi: 10.1371/journal.pone.0242679. PMID: 33237923Free PMC Article
ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: clinicopathologic study of 11 cases and review of the literature.
Maragliano R, Vanoli A, Albarello L, Milione M, Basturk O, Klimstra DS, Wachtel A, Uccella S, Vicari E, Milesi M, Davì MV, Scarpa A, Sessa F, Capella C, La Rosa S
Am J Surg Pathol 2015 Mar;39(3):374-82. doi: 10.1097/PAS.0000000000000340. PMID: 25353285

Recent systematic reviews

PET imaging in ectopic Cushing syndrome: a systematic review.
Santhanam P, Taieb D, Giovanella L, Treglia G
Endocrine 2015 Nov;50(2):297-305. Epub 2015 Jul 25 doi: 10.1007/s12020-015-0689-4. PMID: 26206753

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