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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
Prevalence and Clinical Manifestations of Primary Aldosteronism Encountered in Primary Care Practice.
Monticone S, Burrello J, Tizzani D, Bertello C, Viola A, Buffolo F, Gabetti L, Mengozzi G, Williams TA, Rabbia F, Veglio F, Mulatero P
J Am Coll Cardiol 2017 Apr 11;69(14):1811-1820. doi: 10.1016/j.jacc.2017.01.052. PMID: 28385310

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
Genetic disorders in primary aldosteronism-familial and somatic.
Funder JW
J Steroid Biochem Mol Biol 2017 Jan;165(Pt A):154-157. Epub 2016 Mar 21 doi: 10.1016/j.jsbmb.2016.03.020. PMID: 27013018
Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.
Amar L, Lorthioir A, Azizi M, Plouin PF
Eur J Endocrinol 2015 Mar;172(3):R125-9. Epub 2014 Oct 14 doi: 10.1530/EJE-14-0585. PMID: 25315875

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
Targeting CXCR4 (CXC Chemokine Receptor Type 4) for Molecular Imaging of Aldosterone-Producing Adenoma.
Heinze B, Fuss CT, Mulatero P, Beuschlein F, Reincke M, Mustafa M, Schirbel A, Deutschbein T, Williams TA, Rhayem Y, Quinkler M, Rayes N, Monticone S, Wild V, Gomez-Sanchez CE, Reis AC, Petersenn S, Wester HJ, Kropf S, Fassnacht M, Lang K, Herrmann K, Buck AK, Bluemel C, Hahner S
Hypertension 2018 Feb;71(2):317-325. Epub 2017 Dec 26 doi: 10.1161/HYPERTENSIONAHA.117.09975. PMID: 29279316
Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.
Amar L, Lorthioir A, Azizi M, Plouin PF
Eur J Endocrinol 2015 Mar;172(3):R125-9. Epub 2014 Oct 14 doi: 10.1530/EJE-14-0585. PMID: 25315875
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
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
Hypokalemia and the Prevalence of Primary Aldosteronism.
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
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
Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.
Amar L, Lorthioir A, Azizi M, Plouin PF
Eur J Endocrinol 2015 Mar;172(3):R125-9. Epub 2014 Oct 14 doi: 10.1530/EJE-14-0585. PMID: 25315875

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
Targeting CXCR4 (CXC Chemokine Receptor Type 4) for Molecular Imaging of Aldosterone-Producing Adenoma.
Heinze B, Fuss CT, Mulatero P, Beuschlein F, Reincke M, Mustafa M, Schirbel A, Deutschbein T, Williams TA, Rhayem Y, Quinkler M, Rayes N, Monticone S, Wild V, Gomez-Sanchez CE, Reis AC, Petersenn S, Wester HJ, Kropf S, Fassnacht M, Lang K, Herrmann K, Buck AK, Bluemel C, Hahner S
Hypertension 2018 Feb;71(2):317-325. Epub 2017 Dec 26 doi: 10.1161/HYPERTENSIONAHA.117.09975. PMID: 29279316
Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.
Amar L, Lorthioir A, Azizi M, Plouin PF
Eur J Endocrinol 2015 Mar;172(3):R125-9. Epub 2014 Oct 14 doi: 10.1530/EJE-14-0585. PMID: 25315875

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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