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Aldosterone-producing adrenal cortex adenoma

MedGen UID:
353374
Concept ID:
C1706762
Neoplastic Process
Synonyms: adrenocortical carcinoma with pure aldosterone hypersecretion; Adrenocortical carcinoma with pure aldosterone hypersecretion; Aldosterone Producing Adrenal Cortex Adenoma; aldosterone producing adrenal cortex adenoma; Aldosterone Producing Adrenal Cortical Adenoma; aldosterone producing adrenal cortical adenoma; Aldosterone-Producing Adrenal Cortex Adenoma; aldosterone-producing adrenal cortex adenoma; Aldosterone-Producing Adrenal Cortical Adenoma; Aldosterone-Producing Adrenal Gland Adenoma; APAC; Pure aldosterone-producing adrenocortical carcinoma; Pure aldosterone-secreting adrenocortical carcinoma; Pure APAC
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016505
Orphanet: ORPHA231625

Definition

An aldosterone-producing adenoma is a noncancerous (benign) tumor that develops in an adrenal gland, which is a small hormone-producing gland located on top of each kidney. In most cases, individuals develop a single tumor in one of the adrenal glands. The adrenal tumor produces too much of the hormone aldosterone, which is a condition known as primary hyperaldosteronism. Aldosterone helps regulate the body's fluid levels and blood pressure by controlling the amount of salt retained by the kidneys. Excess aldosterone causes the kidneys to retain more salt than normal, which increases the body's fluid levels and blood pressure. People with an aldosterone-producing adenoma may develop severe high blood pressure (hypertension), and they have an increased risk of heart attack, stroke, or an irregular heart beat (atrial fibrillation). [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAldosterone-producing adrenal cortex adenoma

Professional guidelines

PubMed

Gong S, Sun N, Meyer LS, Tetti M, Koupourtidou C, Krebs S, Masserdotti G, Blum H, Rainey WE, Reincke M, Walch A, Williams TA
Hypertension 2023 Jul;80(7):1555-1567. Epub 2023 May 1 doi: 10.1161/HYPERTENSIONAHA.123.20921. PMID: 37125608Free PMC Article
Reincke M, Bancos I, Mulatero P, Scholl UI, Stowasser M, Williams TA
Lancet Diabetes Endocrinol 2021 Dec;9(12):876-892. doi: 10.1016/S2213-8587(21)00210-2. PMID: 34798068
Yang Y, Reincke M, Williams TA
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101365. Epub 2019 Dec 5 doi: 10.1016/j.beem.2019.101365. PMID: 31837980

Recent clinical studies

Etiology

Ekman N, Grossman AB, Dworakowska D
Int J Mol Sci 2024 Jan 11;25(2) doi: 10.3390/ijms25020900. PMID: 38255973Free PMC Article
Azizan EAB, Drake WM, Brown MJ
Nat Rev Nephrol 2023 Dec;19(12):788-806. Epub 2023 Aug 23 doi: 10.1038/s41581-023-00753-6. PMID: 37612380Free PMC Article
Kitamoto T, Nishikawa T
Int J Mol Sci 2022 Aug 12;23(16) doi: 10.3390/ijms23169042. PMID: 36012306Free PMC Article
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Gordon RD
J Endocrinol Invest 1995 Jul-Aug;18(7):495-511. doi: 10.1007/BF03349761. PMID: 9221268

Diagnosis

Bechmann N, Moskopp ML, Constantinescu G, Stell A, Ernst A, Berthold F, Westermann F, Jiang J, Lui L, Nowak E, Zopp S, Pacak K, Peitzsch M, Schedl A, Reincke M, Beuschlein F, Bornstein SR, Fassnacht M, Eisenhofer G
J Clin Endocrinol Metab 2024 Jan 18;109(2):471-482. doi: 10.1210/clinem/dgad515. PMID: 37647861Free PMC Article
Azizan EAB, Drake WM, Brown MJ
Nat Rev Nephrol 2023 Dec;19(12):788-806. Epub 2023 Aug 23 doi: 10.1038/s41581-023-00753-6. PMID: 37612380Free PMC Article
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Reincke M, Bancos I, Mulatero P, Scholl UI, Stowasser M, Williams TA
Lancet Diabetes Endocrinol 2021 Dec;9(12):876-892. doi: 10.1016/S2213-8587(21)00210-2. PMID: 34798068
Hellman P, Björklund P, Åkerström T
Vitam Horm 2019;109:407-431. Epub 2019 Jan 8 doi: 10.1016/bs.vh.2018.10.007. PMID: 30678866

Therapy

Pilz S, Kocjan T, Theiler-Schwetz V, Trummer C
Pol Arch Intern Med 2023 Oct 26;133(10) Epub 2023 Oct 11 doi: 10.20452/pamw.16585. PMID: 37818947
Deinum J, Groenewoud H, van der Wilt GJ, Lenzini L, Rossi GP
Eur J Endocrinol 2019 Sep;181(3):D15-D26. doi: 10.1530/EJE-18-0844. PMID: 31176302
Inoue K, Yamazaki Y, Kitamoto T, Hirose R, Saito J, Omura M, Sasano H, Nishikawa T
J Clin Endocrinol Metab 2018 Sep 1;103(9):3477-3485. doi: 10.1210/jc.2018-00738. PMID: 30020487
Weiner ID
Semin Nephrol 2013 May;33(3):265-76. doi: 10.1016/j.semnephrol.2013.04.007. PMID: 23953804Free PMC Article
Hegstad R, Brown RD, Jiang NS, Kao P, Weinshilboum RM, Strong C, Wisgerhof M
Am J Med 1983 Mar;74(3):442-8. doi: 10.1016/0002-9343(83)90971-3. PMID: 6338717

Prognosis

Bechmann N, Moskopp ML, Constantinescu G, Stell A, Ernst A, Berthold F, Westermann F, Jiang J, Lui L, Nowak E, Zopp S, Pacak K, Peitzsch M, Schedl A, Reincke M, Beuschlein F, Bornstein SR, Fassnacht M, Eisenhofer G
J Clin Endocrinol Metab 2024 Jan 18;109(2):471-482. doi: 10.1210/clinem/dgad515. PMID: 37647861Free PMC Article
Santana LS, Guimaraes AG, Almeida MQ
Front Endocrinol (Lausanne) 2022;13:927669. Epub 2022 Jun 23 doi: 10.3389/fendo.2022.927669. PMID: 35813615Free PMC Article
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Gruber S, Beuschlein F
Horm Metab Res 2020 Jun;52(6):347-356. Epub 2020 Apr 6 doi: 10.1055/a-1134-4980. PMID: 32252108
Åkerström T, Carling T, Beuschlein F, Hellman P
J Intern Med 2016 Dec;280(6):540-550. doi: 10.1111/joim.12452. PMID: 27864864

Clinical prediction guides

Bechmann N, Moskopp ML, Constantinescu G, Stell A, Ernst A, Berthold F, Westermann F, Jiang J, Lui L, Nowak E, Zopp S, Pacak K, Peitzsch M, Schedl A, Reincke M, Beuschlein F, Bornstein SR, Fassnacht M, Eisenhofer G
J Clin Endocrinol Metab 2024 Jan 18;109(2):471-482. doi: 10.1210/clinem/dgad515. PMID: 37647861Free PMC Article
Santana LS, Guimaraes AG, Almeida MQ
Front Endocrinol (Lausanne) 2022;13:927669. Epub 2022 Jun 23 doi: 10.3389/fendo.2022.927669. PMID: 35813615Free PMC Article
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Lim JS, Plaska SW, Rege J, Rainey WE, Turcu AF
Front Endocrinol (Lausanne) 2021;12:644382. Epub 2021 Mar 16 doi: 10.3389/fendo.2021.644382. PMID: 33796077Free PMC Article
Holler F, Heinrich DA, Adolf C, Lechner B, Bidlingmaier M, Eisenhofer G, Williams TA, Reincke M
Curr Hypertens Rep 2019 Sep 3;21(10):77. doi: 10.1007/s11906-019-0985-0. PMID: 31482378

Recent systematic reviews

Marzano L, Kazory A, Husain-Syed F, Ronco C
Clin Endocrinol (Oxf) 2023 Jul;99(1):17-34. Epub 2023 Apr 20 doi: 10.1111/cen.14918. PMID: 37032125
Velema MS, de Nooijer AH, Burgers VWG, Hermus ARMM, Timmers HJLM, Lenders JWM, Husson O, Deinum J
Horm Metab Res 2017 Dec;49(12):943-950. Epub 2017 Dec 4 doi: 10.1055/s-0043-121706. PMID: 29202493

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