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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Treating Primary Aldosteronism-Induced Hypertension: Novel Approaches and Future Outlooks.
Mullen N, Curneen J, Donlon PT, Prakash P, Bancos I, Gurnell M, Dennedy MC
Endocr Rev 2024 Jan 4;45(1):125-170. doi: 10.1210/endrev/bnad026. PMID: 37556722Free PMC Article
Diagnosis and treatment of primary aldosteronism.
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
Aldosterone-Producing Adenomas: Genotypes and Phenotypes.
Satoh F, Sasano H, Yamazaki Y, Ito S
Hypertension 2017 Jul;70(1):38-41. Epub 2017 Jun 5 doi: 10.1161/HYPERTENSIONAHA.117.09161. PMID: 28584015

Recent clinical studies

Etiology

What We Know about and What Is New in Primary Aldosteronism.
Ekman N, Grossman AB, Dworakowska D
Int J Mol Sci 2024 Jan 11;25(2) doi: 10.3390/ijms25020900. PMID: 38255973Free PMC Article
Primary aldosteronism: molecular medicine meets public health.
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
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
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
Radiofrequency ablation treatment for aldosterone-producing adenomas.
Sacks BA, Sacks AC, Faintuch S
Curr Opin Endocrinol Diabetes Obes 2017 Jun;24(3):169-173. doi: 10.1097/MED.0000000000000329. PMID: 28248752
Primary aldosteronism.
Gordon RD
J Endocrinol Invest 1995 Jul-Aug;18(7):495-511. doi: 10.1007/BF03349761. PMID: 9221268

Diagnosis

What We Know about and What Is New in Primary Aldosteronism.
Ekman N, Grossman AB, Dworakowska D
Int J Mol Sci 2024 Jan 11;25(2) doi: 10.3390/ijms25020900. PMID: 38255973Free PMC Article
Primary aldosteronism: molecular medicine meets public health.
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
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
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
Diagnosis and treatment of primary aldosteronism.
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
Aldosterone-Producing Adenomas.
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

Treating Primary Aldosteronism-Induced Hypertension: Novel Approaches and Future Outlooks.
Mullen N, Curneen J, Donlon PT, Prakash P, Bancos I, Gurnell M, Dennedy MC
Endocr Rev 2024 Jan 4;45(1):125-170. doi: 10.1210/endrev/bnad026. PMID: 37556722Free PMC Article
Postoperative ACTH-stimulated aldosterone predicts biochemical outcome in primary aldosteronism.
Bruedgam D, Adolf C, Schneider H, Schwarzlmueller P, Mueller L, Handgriff L, Bidlingmaier M, Kunz S, Zimmermann P, Deniz S, Williams TA, Beuschlein F, Reincke M, Heinrich DA
Eur J Endocrinol 2023 Dec 6;189(6):611-618. doi: 10.1093/ejendo/lvad159. PMID: 38048424
Adrenal venous sampling: cosyntropin stimulation or not?
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
Primary aldosteronism: management issues.
Young WF Jr
Ann N Y Acad Sci 2002 Sep;970:61-76. doi: 10.1111/j.1749-6632.2002.tb04413.x. PMID: 12381542
Aging and aldosterone.
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

Treating Primary Aldosteronism-Induced Hypertension: Novel Approaches and Future Outlooks.
Mullen N, Curneen J, Donlon PT, Prakash P, Bancos I, Gurnell M, Dennedy MC
Endocr Rev 2024 Jan 4;45(1):125-170. doi: 10.1210/endrev/bnad026. PMID: 37556722Free PMC Article
Pathogenesis of Primary Aldosteronism: Impact on Clinical Outcome.
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
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
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
Genetics of adrenocortical tumours.
Åkerström T, Carling T, Beuschlein F, Hellman P
J Intern Med 2016 Dec;280(6):540-550. doi: 10.1111/joim.12452. PMID: 27864864
Management of adrenal incidentaloma.
Terzolo M, Bovio S, Pia A, Reimondo G, Angeli A
Best Pract Res Clin Endocrinol Metab 2009 Apr;23(2):233-43. doi: 10.1016/j.beem.2009.04.001. PMID: 19500766

Clinical prediction guides

Single-Nucleus Analysis Reveals Tumor Heterogeneity of Aldosterone-Producing Adenoma.
Murakami M, Hara K, Ikeda K, Horino M, Okazaki R, Niitsu Y, Takeuchi A, Aoki J, Shiba K, Tsujimoto K, Komiya C, Nakamura Y, Kurata M, Akashi T, Fujii Y, Yamada T
Hypertension 2024 Feb;81(2):361-371. Epub 2023 Dec 14 doi: 10.1161/HYPERTENSIONAHA.123.21446. PMID: 38095094
Treating Primary Aldosteronism-Induced Hypertension: Novel Approaches and Future Outlooks.
Mullen N, Curneen J, Donlon PT, Prakash P, Bancos I, Gurnell M, Dennedy MC
Endocr Rev 2024 Jan 4;45(1):125-170. doi: 10.1210/endrev/bnad026. PMID: 37556722Free PMC Article
Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.
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
Targeted Mutational Analysis of Cortisol-Producing Adenomas.
Rege J, Hoxie J, Liu CJ, Cash MN, Luther JM, Gellert L, Turcu AF, Else T, Giordano TJ, Udager AM, Rainey WE, Nanba K
J Clin Endocrinol Metab 2022 Jan 18;107(2):e594-e603. doi: 10.1210/clinem/dgab682. PMID: 34534321Free PMC Article
Aldosterone-Regulating Receptors and Aldosterone-Driver Somatic Mutations.
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

Recent systematic reviews

Cardiovascular Outcomes of KCNJ5 Mutated Aldosterone-Producing Adenoma: A Systematic Review.
Kato H, Kitamoto T, Kimura S, Sunouchi T, Hoshino Y, Hidaka N, Tsurutani Y, Ito N, Makita N, Nishikawa T, Nangaku M, Inoue K
Endocr Pract 2024 Jul;30(7):670-678. Epub 2024 Apr 22 doi: 10.1016/j.eprac.2024.04.007. PMID: 38657793
Prognostic models to predict complete resolution of hypertension after adrenalectomy in primary aldosteronism: A systematic review and meta-analysis.
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
Health-Related Quality of Life and Mental Health in Primary Aldosteronism: A Systematic Review.
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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