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Pigmented Adrenal Cortical Adenoma

MedGen UID:
137744
•Concept ID:
C0334333
•
Neoplastic Process
Synonyms: Adrenal cortical adenoma - heavily pigmented variant; Adrenal cortical adenoma, heavily pigmented variant; Adrenal cortical adenoma, pigmented; Black adenoma; Black Adenoma; Heavily Pigmented Adrenal Cortex Adenoma; Heavily Pigmented Adrenal Cortical Adenoma; Heavily Pigmented Adrenocortical Adenoma; Pigmented adenoma; Pigmented Adrenal Cortex Adenoma; Pigmented Adrenal Gland Adenoma
SNOMED CT: Adrenal cortical adenoma - heavily pigmented variant (37302003); Pigmented adenoma (37302003); Adrenal cortical adenoma, pigmented (37302003); Adrenal cortical adenoma, heavily pigmented variant (37302003); Black adenoma (37302003)

Definition

A usually functioning adenoma of the adrenal cortex. Grossly, it has a dark brown appearance and is characterized by the presence of neoplastic cells containing abundant intracytoplasmic lipofuscin. It may be associated with Cushing syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPigmented Adrenal Cortical Adenoma

Professional guidelines

PubMed

Surgical management of Cushing's syndrome.
Imai T, Kikumori T, Funahashi H, Nakao A
Biomed Pharmacother 2000 Jun;54 Suppl 1:140s-145s. doi: 10.1016/s0753-3322(00)80031-1. PMID: 10915011
Adrenalectomy for treatment of Cushing syndrome: results in 122 patients and long-term follow-up studies.
Imai T, Funahashi H, Tanaka Y, Tobinaga J, Wada M, Morita-Matsuyama T, Ohiso Y, Takagi H
World J Surg 1996 Sep;20(7):781-6; discussion 786-7. doi: 10.1007/s002689900119. PMID: 8678951
Primary adrenal causes of Cushing's syndrome. Diagnosis and surgical management.
Perry RR, Nieman LK, Cutler GB Jr, Chrousos GP, Loriaux DL, Doppman JL, Travis WD, Norton JA
Ann Surg 1989 Jul;210(1):59-68. doi: 10.1097/00000658-198907000-00010. PMID: 2742414Free PMC Article

Recent clinical studies

Etiology

Predicting the risk of cardiac myxoma in Carney complex.
Pitsava G, Zhu C, Sundaram R, Mills JL, Stratakis CA
Genet Med 2021 Jan;23(1):80-85. Epub 2020 Sep 7 doi: 10.1038/s41436-020-00956-3. PMID: 32893266Free PMC Article
Adrenalectomy for non-neuroblastic pathology in children.
Traynor MD Jr, Sada A, Thompson GB, Moir CR, Bancos I, Farley DR, Dy BM, Lyden ML, Habermann EB, McKenzie TJ
Pediatr Surg Int 2020 Feb;36(2):129-135. Epub 2019 Nov 5 doi: 10.1007/s00383-019-04589-9. PMID: 31691026
Diurnal Plasma Cortisol Measurements Utility in Differentiating Various Etiologies of Endogenous Cushing Syndrome.
Tirosh A, Lodish MB, Papadakis GZ, Lyssikatos C, Belyavskaya E, Stratakis CA
Horm Metab Res 2016 Sep;48(10):677-681. Epub 2016 Sep 19 doi: 10.1055/s-0042-115644. PMID: 27643448Free PMC Article
Surgical management of Cushing's syndrome.
Imai T, Kikumori T, Funahashi H, Nakao A
Biomed Pharmacother 2000 Jun;54 Suppl 1:140s-145s. doi: 10.1016/s0753-3322(00)80031-1. PMID: 10915011
Cushing's syndrome in childhood and adolescence.
Robyn JA, Koch CA, Montalto J, Yong A, Warne GL, Batch JA
J Paediatr Child Health 1997 Dec;33(6):522-7. doi: 10.1111/j.1440-1754.1997.tb01663.x. PMID: 9484685

Diagnosis

Adrenal Cushing's syndrome in children.
Guarnotta V, Emanuele F, Salzillo R, Giordano C
Front Endocrinol (Lausanne) 2023;14:1329082. Epub 2023 Dec 12 doi: 10.3389/fendo.2023.1329082. PMID: 38192416Free PMC Article
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670
Cushing's disease.
Bertagna X, Guignat L, Groussin L, Bertherat J
Best Pract Res Clin Endocrinol Metab 2009 Oct;23(5):607-23. doi: 10.1016/j.beem.2009.06.001. PMID: 19945026
Rare syndromes.
Jabbour SA, Davidovici BB, Wolf R
Clin Dermatol 2006 Jul-Aug;24(4):299-316. doi: 10.1016/j.clindermatol.2006.04.005. PMID: 16828412
Familial endocrine myxolentiginosis.
Panossian DH, Marais GE, Marais HJ
Clin Cardiol 1995 Nov;18(11):675-8. doi: 10.1002/clc.4960181116. PMID: 8590539

Therapy

Predicting the risk of cardiac myxoma in Carney complex.
Pitsava G, Zhu C, Sundaram R, Mills JL, Stratakis CA
Genet Med 2021 Jan;23(1):80-85. Epub 2020 Sep 7 doi: 10.1038/s41436-020-00956-3. PMID: 32893266Free PMC Article
A case of autonomous cortisol secretion in a patient with subclinical Cushing's syndrome, GNAS mutation, and paradoxical cortisol response to dexamethasone.
Sakaguchi C, Ashida K, Kohashi K, Ohe K, Fujii Y, Yano S, Matsuda Y, Sakamoto S, Sakamoto R, Oda Y, Nomura M, Ogawa Y
BMC Endocr Disord 2019 Jan 22;19(1):13. doi: 10.1186/s12902-019-0345-8. PMID: 30670014Free PMC Article
Circadian Plasma Cortisol Measurements Reflect Severity of Hypercortisolemia in Children with Different Etiologies of Endogenous Cushing Syndrome.
Tirosh A, Lodish MB, Lyssikatos C, Belyavskaya E, Papadakis GZ, Stratakis CA
Horm Res Paediatr 2017;87(5):295-300. Epub 2017 Apr 21 doi: 10.1159/000464463. PMID: 28433999Free PMC Article
Surgical management of Cushing's syndrome.
Imai T, Kikumori T, Funahashi H, Nakao A
Biomed Pharmacother 2000 Jun;54 Suppl 1:140s-145s. doi: 10.1016/s0753-3322(00)80031-1. PMID: 10915011
Adrenalectomy for treatment of Cushing syndrome: results in 122 patients and long-term follow-up studies.
Imai T, Funahashi H, Tanaka Y, Tobinaga J, Wada M, Morita-Matsuyama T, Ohiso Y, Takagi H
World J Surg 1996 Sep;20(7):781-6; discussion 786-7. doi: 10.1007/s002689900119. PMID: 8678951

Prognosis

Predicting the risk of cardiac myxoma in Carney complex.
Pitsava G, Zhu C, Sundaram R, Mills JL, Stratakis CA
Genet Med 2021 Jan;23(1):80-85. Epub 2020 Sep 7 doi: 10.1038/s41436-020-00956-3. PMID: 32893266Free PMC Article
Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.
Katanić D, Kafka D, Živojinov M, Vlaški J, Budakov Z, Knežević Pogančev M, Vorgučin I, Ćuk T
J Pediatr Endocrinol Metab 2017 May 1;30(5):603-609. doi: 10.1515/jpem-2016-0249. PMID: 28391254
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670
Cushing's disease.
Bertagna X, Guignat L, Groussin L, Bertherat J
Best Pract Res Clin Endocrinol Metab 2009 Oct;23(5):607-23. doi: 10.1016/j.beem.2009.06.001. PMID: 19945026
Oncocytic adrenal cortical adenoma.
Erlandson RA, Reuter VE
Ultrastruct Pathol 1991 Jul-Oct;15(4-5):539-47. doi: 10.3109/01913129109016261. PMID: 1721751

Clinical prediction guides

Clinical Spectrum of Adrenal Cushing's Syndrome and the Caution for Interpretation of Adrenocorticotrophic Hormone: A Single-Center Experience.
Gosavi V, Lila A, Memon SS, Sarathi V, Thakkar K, Dalvi A, Malhotra G, Prakash G, Patil V, Shah NS, Bandgar T
Horm Metab Res 2022 Feb;54(2):57-66. Epub 2022 Feb 7 doi: 10.1055/a-1735-3232. PMID: 35130566
Predicting the risk of cardiac myxoma in Carney complex.
Pitsava G, Zhu C, Sundaram R, Mills JL, Stratakis CA
Genet Med 2021 Jan;23(1):80-85. Epub 2020 Sep 7 doi: 10.1038/s41436-020-00956-3. PMID: 32893266Free PMC Article
Pathology of the adrenal cortex: a reappraisal of the past 25 years focusing on adrenal cortical tumors.
Papotti M, Duregon E, Volante M, McNicol AM
Endocr Pathol 2014 Mar;25(1):35-48. doi: 10.1007/s12022-013-9291-6. PMID: 24382573
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670
Oncocytic adrenal cortical adenoma.
Erlandson RA, Reuter VE
Ultrastruct Pathol 1991 Jul-Oct;15(4-5):539-47. doi: 10.3109/01913129109016261. PMID: 1721751

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