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Adrenal rest tumor

MedGen UID:
7902
•Concept ID:
C0001630
•
Neoplastic Process
Synonyms: Adrenal Cortical Rest Tumor; Adrenal Rest Tumor; Adrenal Rest Tumors; Rest Tumor, Adrenal; Rest Tumors, Adrenal; Tumor, Adrenal Rest; Tumors, Adrenal Rest
SNOMED CT: Adrenal rest tumor (54292009)
 
Monarch Initiative: MONDO:0002110

Definition

A benign, testicular or ovarian tumor, derived from adrenal embryonic rest cells. It is composed of hyperplastic adrenal cortical tissue, and it is associated with congenital adrenal hyperplasia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenal rest tumor

Professional guidelines

PubMed

Infertility risk assessment with ultrasound in congenital adrenal hyperplasia male patients.
Wang Z, Wang R, Wang X, Zheng S, Li M, Yu Y, Liu Z, Sun S, Zhan W
Sci Rep 2024 May 27;14(1):12058. doi: 10.1038/s41598-024-62954-8. PMID: 38802468Free PMC Article
Genotype of congenital adrenal hyperplasia patients with testicular adrenal rest tumor.
Aycan Z, Keskin M, Lafcı NG, Savaş-Erdeve Ş, Baş F, Poyrazoğlu Ş, Öztürk P, Parlak M, Ercan O, Güran T, Hatipoğlu N, Uçaktürk SA, Çatlı G, Akyürek N, Önder A, Kılınç S, Çetinkaya S
Eur J Med Genet 2022 Dec;65(12):104654. Epub 2022 Nov 4 doi: 10.1016/j.ejmg.2022.104654. PMID: 36343887
Management considerations for the adult with congenital adrenal hyperplasia.
Auchus RJ
Mol Cell Endocrinol 2015 Jun 15;408:190-7. Epub 2015 Jan 30 doi: 10.1016/j.mce.2015.01.039. PMID: 25643980

Recent clinical studies

Etiology

Hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia: a retrospective multicenter cohort study.
Schröder MAM, Neacşu M, Adriaansen BPH, Sweep FCGJ, Ahmed SF, Ali SR, Bachega TASS, Baronio F, Birkebæk NH, de Bruin C, Bonfig W, Bryce J, Clemente M, Cools M, Elsedfy H, Globa E, Guran T, Güven A, Amr NH, Janus D, Taube NL, Markosyan R, Miranda M, Poyrazoğlu Ş, Rees A, Salerno M, Stancampiano MR, Vieites A, de Vries L, Yavas Abali Z, Span PN, Claahsen-van der Grinten HL
Eur J Endocrinol 2023 Oct 17;189(4):460-468. doi: 10.1093/ejendo/lvad143. PMID: 37837609
Risk factors for testicular adrenal rest tumors in pediatric patients with congenital adrenal hyperplasia.
Rivera-Hernandez A, Jimenez-Osorio M, Rodríguez-Mencias JP, Escamilla-Castañeda KM, Madrigal-Gonzalez MM, Zurita-Cruz J
J Pediatr Urol 2023 Aug;19(4):398.e1-398.e7. Epub 2023 Mar 24 doi: 10.1016/j.jpurol.2023.03.028. PMID: 37029011
Patient and Parent Perspectives on Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia.
Nebesio TD, Kim MS, Szymanski KM, Kokorowski PJ, Geffner ME, Eugster EA; Life with Congenital Adrenal Hyperplasia Study Group
Horm Res Paediatr 2023;96(5):518-522. Epub 2023 Jan 18 doi: 10.1159/000529211. PMID: 36652933
Adrenal rest tumours in congenital adrenal hyperplasia.
Zöllner EW, Pitcher R
S Afr Med J 2007 Dec;97(12):1254. PMID: 18271117
Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo and in vitro studies).
Bercovici JP, Fiet J, Gibault L, Volant A, Abalain JH, Floch HH, Sonnet E, Fournier G
J Steroid Biochem Mol Biol 2005 Jan;93(1):67-72. Epub 2005 Jan 26 doi: 10.1016/j.jsbmb.2004.10.023. PMID: 15748834

Diagnosis

Testicular adrenal rest tumors - Epidemiology, diagnosis and treatment.
Eyer de Jesus L, Paz de Oliveira AP, Porto LC, Dekermacher S
J Pediatr Urol 2024 Feb;20(1):77-87. Epub 2023 Oct 7 doi: 10.1016/j.jpurol.2023.10.005. PMID: 37845103
Adult Hypogonadism Due to Testicular Adrenal Rest Tumors.
Yamamoto K, Oka K, Hasegawa K, Otsuka F
Intern Med 2021 Dec 1;60(23):3839-3840. Epub 2021 Jun 19 doi: 10.2169/internalmedicine.6501-20. PMID: 34148948Free PMC Article
Testicular adrenal rest tumor without congenital adrenal hyperplasia: A rare case.
Osaka A, Ide H, Ono Y, Arai G, Ueda Y, Okada H
Int J Urol 2018 Oct;25(10):903-904. Epub 2018 Jul 24 doi: 10.1111/iju.13766. PMID: 30041282
Benign intrascrotal lesions.
Rubenstein RA, Dogra VS, Seftel AD, Resnick MI
J Urol 2004 May;171(5):1765-72. doi: 10.1097/01.ju.0000123083.98845.88. PMID: 15076274
Ovarian tumors. A review.
Scully RE
Am J Pathol 1977 Jun;87(3):686-720. PMID: 194486Free PMC Article

Therapy

Long-term Complications and Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia.
Kılınç Uğurlu A, Özsu E, Aycan Z
J Clin Res Pediatr Endocrinol 2025 Jan 10;17(Suppl 1):60-65. Epub 2024 Dec 23 doi: 10.4274/jcrpe.galenos.2024.2024-6-22-S. PMID: 39713888Free PMC Article
Testicular adrenal rest tumors.
Armas-Alvarez AL, Salinas-Sánchez AS, Atienzar-Tobarra M, Virseda-Rodriguez JA
Rev Int Androl 2018 Jul-Sep;16(3):128-130. Epub 2018 Mar 2 doi: 10.1016/j.androl.2017.10.001. PMID: 30300134
Reversal of primary male infertility and testicular adrenal rest tumors in salt-wasting congenital adrenal hyperplasia.
Collet TH, Pralong FP
J Clin Endocrinol Metab 2010 May;95(5):2013-4. doi: 10.1210/jc.2009-2691. PMID: 20444929
In vivo endocrine studies in adrenal rest tumor of ovary.
Parker CR Jr, Servy E, McDonough PG, Mahesh VB
Obstet Gynecol 1974 Sep;44(3):327-32. PMID: 4277531
Ovarian adrenal rest tumor (masculinovoblastoma) simulating adrenal virilism; report of a case with hormone studies.
EPSTEIN JA, LEVINSON CJ, KUPPERMAN HS
Am J Obstet Gynecol 1957 Nov;74(5):982-8. doi: 10.1016/0002-9378(57)90141-2. PMID: 13469882

Prognosis

Testicular adrenal rest tumors - Epidemiology, diagnosis and treatment.
Eyer de Jesus L, Paz de Oliveira AP, Porto LC, Dekermacher S
J Pediatr Urol 2024 Feb;20(1):77-87. Epub 2023 Oct 7 doi: 10.1016/j.jpurol.2023.10.005. PMID: 37845103
Hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia: a retrospective multicenter cohort study.
Schröder MAM, Neacşu M, Adriaansen BPH, Sweep FCGJ, Ahmed SF, Ali SR, Bachega TASS, Baronio F, Birkebæk NH, de Bruin C, Bonfig W, Bryce J, Clemente M, Cools M, Elsedfy H, Globa E, Guran T, Güven A, Amr NH, Janus D, Taube NL, Markosyan R, Miranda M, Poyrazoğlu Ş, Rees A, Salerno M, Stancampiano MR, Vieites A, de Vries L, Yavas Abali Z, Span PN, Claahsen-van der Grinten HL
Eur J Endocrinol 2023 Oct 17;189(4):460-468. doi: 10.1093/ejendo/lvad143. PMID: 37837609
Bilateral testicular masses and adrenal insufficiency: is congenital adrenal hyperplasia the only possible diagnosis? First two cases of TARTS described in Addison-only X-linked adrenoleukodystrophy and a brief review of literature.
Tresoldi AS, Betella N, Hasenmajer V, Pozza C, Vena W, Fiamengo B, Negri L, Cappa M, Lania AGA, Lenzi A, Isidori AM, Pizzocaro A
J Endocrinol Invest 2021 Mar;44(3):391-402. Epub 2020 Jul 20 doi: 10.1007/s40618-020-01362-x. PMID: 32691371
Adrenal rest tumours in congenital adrenal hyperplasia.
Zöllner EW, Pitcher R
S Afr Med J 2007 Dec;97(12):1254. PMID: 18271117
Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo and in vitro studies).
Bercovici JP, Fiet J, Gibault L, Volant A, Abalain JH, Floch HH, Sonnet E, Fournier G
J Steroid Biochem Mol Biol 2005 Jan;93(1):67-72. Epub 2005 Jan 26 doi: 10.1016/j.jsbmb.2004.10.023. PMID: 15748834

Clinical prediction guides

Hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia: a retrospective multicenter cohort study.
Schröder MAM, Neacşu M, Adriaansen BPH, Sweep FCGJ, Ahmed SF, Ali SR, Bachega TASS, Baronio F, Birkebæk NH, de Bruin C, Bonfig W, Bryce J, Clemente M, Cools M, Elsedfy H, Globa E, Guran T, Güven A, Amr NH, Janus D, Taube NL, Markosyan R, Miranda M, Poyrazoğlu Ş, Rees A, Salerno M, Stancampiano MR, Vieites A, de Vries L, Yavas Abali Z, Span PN, Claahsen-van der Grinten HL
Eur J Endocrinol 2023 Oct 17;189(4):460-468. doi: 10.1093/ejendo/lvad143. PMID: 37837609
Risk factors for testicular adrenal rest tumors in pediatric patients with congenital adrenal hyperplasia.
Rivera-Hernandez A, Jimenez-Osorio M, Rodríguez-Mencias JP, Escamilla-Castañeda KM, Madrigal-Gonzalez MM, Zurita-Cruz J
J Pediatr Urol 2023 Aug;19(4):398.e1-398.e7. Epub 2023 Mar 24 doi: 10.1016/j.jpurol.2023.03.028. PMID: 37029011
Morphologic and Molecular Characterization of Adrenals and Adrenal Rest Affected by Congenital Adrenal Hyperplasia.
Kolli V, da Cunha IW, Kim S, Iben JR, Mallappa A, Li T, Gaynor A, Coon SL, Quezado MM, Merke DP
Front Endocrinol (Lausanne) 2021;12:730947. Epub 2021 Sep 20 doi: 10.3389/fendo.2021.730947. PMID: 34616364Free PMC Article
Testicular adrenal rest tumors in children with congenital adrenal hyperplasia.
Al-Ghamdi WM, Shazly MA, Al-Agha AE
Saudi Med J 2021 Sep;42(9):986-993. doi: 10.15537/smj.2021.42.9.20210257. PMID: 34470837Free PMC Article
Nonfunctioning adrenocortical carcinoma.
Lele SM, Mittal BV, Vora IM, Kulkarni HS
J Postgrad Med 1992 Jul-Sep;38(3):145-7. PMID: 1303421

Recent systematic reviews

Congenital adrenal hyperplasia, disorders of sex development, and infertility in patients with POR gene pathogenic variants: a systematic review of the literature.
Gusmano C, Cannarella R, Crafa A, Barbagallo F, La Vignera S, Condorelli RA, Calogero AE
J Endocrinol Invest 2023 Jan;46(1):1-14. Epub 2022 Jul 17 doi: 10.1007/s40618-022-01849-9. PMID: 35842891Free PMC Article

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