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Testicular adrenal rest tumor

MedGen UID:
1381855
•Concept ID:
C4476786
•
Neoplastic Process
Synonym: Testicular adrenal rest tumors
 
HPO: HP:0025451

Definition

Testicular adrenal rest tumor (TART) is a abenign tumor of the testis. TART generally occurs multiply and bilaterally within the rete testis. Histologically, TART resemble adrenocortical tissue, which led to the name. The tumous are not encapsulated and consist of sheets or confluent cords of large polygonal cells with abundant eosinophilic cytoplasm. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTesticular adrenal rest tumor

Professional guidelines

PubMed

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
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
Prevalence of Testicular Adrenal Rest Tumor and Factors Associated with Its Development in Congenital Adrenal Hyperplasia.
Mendes-Dos-Santos CT, Martins DL, Guerra-Júnior G, Baptista MTM, de-Mello MP, de Oliveira LC, Morcillo AM, Lemos-Marini SHV
Horm Res Paediatr 2018;90(3):161-168. Epub 2018 Aug 27 doi: 10.1159/000492082. PMID: 30149373
Testicular adrenal rest tumor screening and fertility counseling among males with congenital adrenal hyperplasia.
Chaudhari M, Johnson EK, DaJusta D, Nahata L
J Pediatr Urol 2018 Apr;14(2):155.e1-155.e6. Epub 2017 Dec 21 doi: 10.1016/j.jpurol.2017.11.011. PMID: 29330018
Testicular adrenal rest tumors in congenital adrenal hyperplasia-cross-sectional study of 51 Croatian male patients.
Dumic M, Duspara V, Grubic Z, Oguic SK, Skrabic V, Kusec V
Eur J Pediatr 2017 Oct;176(10):1393-1404. Epub 2017 Sep 6 doi: 10.1007/s00431-017-3008-7. PMID: 28879515

Diagnosis

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
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
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
Testicular adrenal rest tumor screening and fertility counseling among males with congenital adrenal hyperplasia.
Chaudhari M, Johnson EK, DaJusta D, Nahata L
J Pediatr Urol 2018 Apr;14(2):155.e1-155.e6. Epub 2017 Dec 21 doi: 10.1016/j.jpurol.2017.11.011. PMID: 29330018
Testicular adrenal rest tumors in congenital adrenal hyperplasia-cross-sectional study of 51 Croatian male patients.
Dumic M, Duspara V, Grubic Z, Oguic SK, Skrabic V, Kusec V
Eur J Pediatr 2017 Oct;176(10):1393-1404. Epub 2017 Sep 6 doi: 10.1007/s00431-017-3008-7. PMID: 28879515

Therapy

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
Combined adrenal failure and testicular adrenal rest tumor in a patient with nicotinamide nucleotide transhydrogenase deficiency.
Hershkovitz E, Arafat M, Loewenthal N, Haim A, Parvari R
J Pediatr Endocrinol Metab 2015 Sep;28(9-10):1187-90. doi: 10.1515/jpem-2015-0075. PMID: 25879317
Total adrenal volume but not testicular adrenal rest tumor volume is associated with hormonal control in patients with 21-hydroxylase deficiency.
Reisch N, Scherr M, Flade L, Bidlingmaier M, Schwarz HP, Müller-Lisse U, Reincke M, Quinkler M, Beuschlein F
J Clin Endocrinol Metab 2010 May;95(5):2065-72. Epub 2010 Feb 26 doi: 10.1210/jc.2009-1929. PMID: 20190160

Prognosis

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
Characterization of the CYP11A1 Nonsynonymous Variant p.E314K in Children Presenting With Adrenal Insufficiency.
Kolli V, Kim H, Torky A, Lao Q, Tatsi C, Mallappa A, Merke DP
J Clin Endocrinol Metab 2019 Feb 1;104(2):269-276. doi: 10.1210/jc.2018-01661. PMID: 30299480Free PMC Article
Testicular adrenal rest tumor screening and fertility counseling among males with congenital adrenal hyperplasia.
Chaudhari M, Johnson EK, DaJusta D, Nahata L
J Pediatr Urol 2018 Apr;14(2):155.e1-155.e6. Epub 2017 Dec 21 doi: 10.1016/j.jpurol.2017.11.011. PMID: 29330018
Combined adrenal failure and testicular adrenal rest tumor in a patient with nicotinamide nucleotide transhydrogenase deficiency.
Hershkovitz E, Arafat M, Loewenthal N, Haim A, Parvari R
J Pediatr Endocrinol Metab 2015 Sep;28(9-10):1187-90. doi: 10.1515/jpem-2015-0075. PMID: 25879317
Total adrenal volume but not testicular adrenal rest tumor volume is associated with hormonal control in patients with 21-hydroxylase deficiency.
Reisch N, Scherr M, Flade L, Bidlingmaier M, Schwarz HP, Müller-Lisse U, Reincke M, Quinkler M, Beuschlein F
J Clin Endocrinol Metab 2010 May;95(5):2065-72. Epub 2010 Feb 26 doi: 10.1210/jc.2009-1929. PMID: 20190160

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
Prevalence, clinical characteristics and long-term outcomes of classical 11 β-hydroxylase deficiency (11BOHD) in Turkish population and novel mutations in CYP11B1 gene.
Baş F, Toksoy G, Ergun-Longmire B, Uyguner ZO, Abalı ZY, Poyrazoğlu Ş, Karaman V, Avcı Ş, Altunoğlu U, Bundak R, Karaman B, Başaran S, Darendeliler F
J Steroid Biochem Mol Biol 2018 Jul;181:88-97. Epub 2018 Apr 4 doi: 10.1016/j.jsbmb.2018.04.001. PMID: 29626607
Testicular Adrenal Rest Tumor (TART) in congenital adrenal hyperplasia.
Ozisik H, Yurekli BS, Simsir IY, Altun I, Soyaltin U, Guler E, Onay H, Sarsik B, Saygili F
Eur J Med Genet 2017 Sep;60(9):489-493. Epub 2017 Jul 1 doi: 10.1016/j.ejmg.2017.06.009. PMID: 28676275
Combined adrenal failure and testicular adrenal rest tumor in a patient with nicotinamide nucleotide transhydrogenase deficiency.
Hershkovitz E, Arafat M, Loewenthal N, Haim A, Parvari R
J Pediatr Endocrinol Metab 2015 Sep;28(9-10):1187-90. doi: 10.1515/jpem-2015-0075. PMID: 25879317
Testicular adrenal rest tumors and Leydig and Sertoli cell function in boys with classical congenital adrenal hyperplasia.
Martinez-Aguayo A, Rocha A, Rojas N, García C, Parra R, Lagos M, Valdivia L, Poggi H, Cattani A; Chilean Collaborative Testicular Adrenal Rest Tumor Study Group
J Clin Endocrinol Metab 2007 Dec;92(12):4583-9. Epub 2007 Sep 25 doi: 10.1210/jc.2007-0383. PMID: 17895312

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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