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Cortisol-producing adrenal cortex adenoma

MedGen UID:: 353568
•Concept ID:: C1707525
•: Neoplastic Process

Synonyms:	Cortisol Producing Adrenal Cortex Adenoma; cortisol producing adrenal cortex adenoma; Cortisol Producing Adrenal Cortical Adenoma; cortisol producing adrenal cortical adenoma; Cortisol-Producing Adrenal Cortex Adenoma; cortisol-producing adrenal cortex adenoma; Cortisol-Producing Adrenal Cortical Adenoma; Cortisol-Producing Adrenal Gland Adenoma

Monarch Initiative:	MONDO:0006174

Definition

An adenoma of the adrenal cortex that produces cortisol. It may be associated with Cushing syndrome. Clinical presentation includes weight gain, round face, easy bruising, muscle weakness, emotional changes, hirsutism, and hypertension. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCortisol-producing adrenal cortex adenoma

Pathological process
- Disease
  - Neoplasm
    - Epithelial neoplasm
      - Adenoma
        Adrenocortical adenoma
        Cortisol-producing adrenal cortex adenoma

Professional guidelines

PubMed

Fluorinated aldosterone synthase (CYP11B2)-inhibitors for differential diagnosis between bilateral and unilateral conditions of primary aldosteronism.

Maier P, Heinze B, Gabor S, Reese S, Hahner S, Schirbel A
Bioorg Med Chem Lett 2023 Nov 15;96:129501. Epub 2023 Oct 5 doi: 10.1016/j.bmcl.2023.129501. PMID: 37804995

Genotype-specific cortisol production associated with Cushing's syndrome adenoma with PRKACA mutations.

Baba R, Oki K, Gomez-Sanchez CE, Otagaki Y, Itcho K, Kobuke K, Kodama T, Nagano G, Ohno H, Yoneda M, Hattori N
Mol Cell Endocrinol 2021 Dec 1;538:111456. Epub 2021 Sep 11 doi: 10.1016/j.mce.2021.111456. PMID: 34520814 Free PMC Article

PRKACA Mutations in Adrenal Adenomas: Genotype/Phenotype Correlations.

Dalmazi GD, Beuschlein F
Horm Metab Res 2017 Apr;49(4):301-306. Epub 2016 Nov 21 doi: 10.1055/s-0042-120416. PMID: 27871112

See all (4)

Recent clinical studies

Etiology

PPARG dysregulation as a potential molecular target in adrenal Cushing's syndrome.

Vetrivel S, Tamburello M, Oßwald A, Zhang R, Khan A, Jung S, Baker JE, Rainey WE, Nowak E, Altieri B, Detomas M, Watts D, Williams TA, Wielockx B, Beuschlein F, Reincke M, Sbiera S, Riester A
Front Endocrinol (Lausanne) 2023;14:1265794. Epub 2023 Nov 30 doi: 10.3389/fendo.2023.1265794. PMID: 38098864 Free PMC Article

Heterogeneous circulating miRNA profiles of PBMAH.

Hara K, Murakami M, Niitsu Y, Takeuchi A, Horino M, Shiba K, Tsujimoto K, Komiya C, Ikeda K, Tsuiki M, Tanabe A, Tanaka T, Yokoyama M, Fujii Y, Naruse M, Yamada T
Front Endocrinol (Lausanne) 2022;13:1073328. Epub 2022 Dec 13 doi: 10.3389/fendo.2022.1073328. PMID: 36583003 Free PMC Article

PRKACA Mutations in Adrenal Adenomas: Genotype/Phenotype Correlations.

Dalmazi GD, Beuschlein F
Horm Metab Res 2017 Apr;49(4):301-306. Epub 2016 Nov 21 doi: 10.1055/s-0042-120416. PMID: 27871112

cAMP signaling in cortisol-producing adrenal adenoma.

Calebiro D, Di Dalmazi G, Bathon K, Ronchi CL, Beuschlein F
Eur J Endocrinol 2015 Oct;173(4):M99-106. Epub 2015 Jul 2 doi: 10.1530/EJE-15-0353. PMID: 26139209

Clinicopathological correlates of adrenal Cushing's syndrome.

Duan K, Gomez Hernandez K, Mete O
J Clin Pathol 2015 Mar;68(3):175-86. Epub 2014 Nov 25 doi: 10.1136/jclinpath-2014-202612. PMID: 25425660

See all (45)

Diagnosis

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: 34534321 Free PMC Article

PRECISION MEDICINE IN ADRENAL DISORDERS: THE NEXT GENERATION.

Ghayee HK, Vinik AI, Pacak K; AACE Adrenal Scientific Committee
Endocr Pract 2017 Jun;23(6):672-679. Epub 2017 Mar 23 doi: 10.4158/EP161716.RA. PMID: 28332880 Free 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

The genetics of adrenocortical tumors.

Espiard S, Bertherat J
Endocrinol Metab Clin North Am 2015 Jun;44(2):311-34. doi: 10.1016/j.ecl.2015.02.004. PMID: 26038203

Clinicopathological correlates of adrenal Cushing's syndrome.

Duan K, Gomez Hernandez K, Mete O
J Clin Pathol 2015 Mar;68(3):175-86. Epub 2014 Nov 25 doi: 10.1136/jclinpath-2014-202612. PMID: 25425660

See all (68)

Therapy

Intracranial hypertension and empty Sella from adrenal adenoma and excessive and prolonged steroid usage: a case report.

Zhao N, Yang W, Li X, Wang L, Feng Y
BMC Endocr Disord 2022 Jan 15;22(1):24. doi: 10.1186/s12902-021-00931-2. PMID: 35033053 Free PMC Article

Case Report: Consecutive Adrenal Cushing's Syndrome and Cushing's Disease in a Patient With Somatic CTNNB1, USP8, and NR3C1 Mutations.

Detomas M, Altieri B, Schlötelburg W, Appenzeller S, Schlaffer S, Coras R, Schirbel A, Wild V, Kroiss M, Sbiera S, Fassnacht M, Deutschbein T
Front Endocrinol (Lausanne) 2021;12:731579. Epub 2021 Aug 20 doi: 10.3389/fendo.2021.731579. PMID: 34489873 Free PMC Article

Systemic therapy of Cushing's syndrome.

Eckstein N, Haas B, Hass MD, Pfeifer V
Orphanet J Rare Dis 2014 Aug 5;9:122. doi: 10.1186/s13023-014-0122-8. PMID: 25091295 Free PMC Article

A case of cortisol producing adrenal adenoma without phenotype of Cushing's syndrome due to impaired 11beta-hydroxysteroid dehydrogenase 1 activity.

Arai H, Kobayashi N, Nakatsuru Y, Masuzaki H, Nambu T, Takaya K, Yamanaka Y, Kondo E, Yamada G, Fujii T, Miura M, Komatsu Y, Kanamoto N, Ariyasu H, Moriyama K, Yasoda A, Nakao K
Endocr J 2008 Aug;55(4):709-15. Epub 2008 May 21 doi: 10.1507/endocrj.k08e-008. PMID: 18493111

Classic and recent etiologies of Cushing's syndrome: diagnosis and therapy.

Beauregard C, Dickstein G, Lacroix A
Treat Endocrinol 2002;1(2):79-94. doi: 10.2165/00024677-200201020-00002. PMID: 15765624

See all (14)

Prognosis

What Did We Learn from the Molecular Biology of Adrenal Cortical Neoplasia? From Histopathology to Translational Genomics.

Juhlin CC, Bertherat J, Giordano TJ, Hammer GD, Sasano H, Mete O
Endocr Pathol 2021 Mar;32(1):102-133. Epub 2021 Feb 3 doi: 10.1007/s12022-021-09667-0. PMID: 33534120

Genomic insights into Cushing syndrome.

Assié G
Ann Endocrinol (Paris) 2018 Jun;79(3):119-122. Epub 2018 May 4 doi: 10.1016/j.ando.2018.03.011. PMID: 29735160

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

ENDOCRINE TUMOURS: The genomics of adrenocortical tumors.

Faillot S, Assie G
Eur J Endocrinol 2016 Jun;174(6):R249-65. Epub 2016 Jan 6 doi: 10.1530/EJE-15-1118. PMID: 26739091

Clinicopathological correlates of adrenal Cushing's syndrome.

Duan K, Gomez Hernandez K, Mete O
J Clin Pathol 2015 Mar;68(3):175-86. Epub 2014 Nov 25 doi: 10.1136/jclinpath-2014-202612. PMID: 25425660

See all (16)

Clinical prediction guides

β-Catenin activation and illicit receptor expression in adrenocortical cells.

Le Mestre J, Thomas M, Duparc C, Val P, Bures C, Tuech JJ, Sabourin JC, Baert-Desurmont S, Lefebvre H, Louiset E
Endocr Relat Cancer 2022 Feb 7;29(3):151-162. doi: 10.1530/ERC-21-0298. PMID: 34935630

Targeted Mutational Analysis of Cortisol-Producing Adenomas.

Pathogenesis of benign unilateral adrenocortical tumors: focus on cAMP/PKA pathway.

Weigand I
Minerva Endocrinol 2019 Mar;44(1):25-32. Epub 2018 Jul 2 doi: 10.23736/S0391-1977.18.02874-2. PMID: 29963826

The genetics of adrenocortical tumors.

Espiard S, Bertherat J
Endocrinol Metab Clin North Am 2015 Jun;44(2):311-34. doi: 10.1016/j.ecl.2015.02.004. PMID: 26038203

Clinicopathological correlates of adrenal Cushing's syndrome.

Duan K, Gomez Hernandez K, Mete O
J Clin Pathol 2015 Mar;68(3):175-86. Epub 2014 Nov 25 doi: 10.1136/jclinpath-2014-202612. PMID: 25425660

See all (43)