Congenital adrenal hyperplasia with an ovarian adrenal rest tumor: a case report

Yıl 2020, Cilt: 6 Sayı: 1, 77 - 82, 04.01.2020

Betül Aydın Buyruk Göknur Yorulmaz Ayşe Nur Değer Medine Nur Kebapçı

https://doi.org/10.18621/eurj.435638

Öz

Adrenal
rest tumors (ART) are extra-adrenal findings of adrenal tissue. Testicular
adrenal rest tumors (TART) are widespread in males with congenital adrenal
hyperplasia (CAH). Ovarian adrenal rest tumours (OART), which form in females
with CAH, are less commonly seen. Reports in literature of OART are extemely
rare. The case is here presented of a 43-year-old female who was diagnosed at
the age of 15 years with CAH and underwent bilateral adrenalectomy at the age
of 37. On presentation at our clinic, 4×2 cm mass was determined on the right
adnexa in the advanced tests and a right-side salpingo-oopherectomy was
performed. The pathology examination reported heterotopic adrenal cortical
tissue. Routine ovarian imaging in females with CAH is not indicated. Although
OART are rarely seen, they should be kept in mind and these patients should be
evaluated radiologically together with laboratory tests. 

Anahtar Kelimeler

Ovarian adrenal rest tumor, congenital adrenal hyperplasia

Kaynakça

• 1. Claahsen-van der Grinten HL, Otten BJ, Takahashi S, Meuleman EJ, Hulsbergen-van de Kaa C, Sweep FC, et al. Testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testissparingsurgery in eight patients. J Clin Endocrinol Metab 2007;92: 612-5.
• 2. Claahsen-van der Grinten HL, Otten BJ, Stikkelbroeck MM, Sweep FC, Hermus AR. Testicular adrenal rest tumours in congenital adrenal hyperplasia. Best Pract Res Clin Endocrinol Metab 2009;23:209-20.
• 3. Stikkelbroeck NM, Otten BJ, Pasic A, Jager GJ, Sweep CG, Noordam K, et al. High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:5721-8.
• 4. Merke DP. Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab 2008;93:653-60.
• 5. Ambroziak U Bednarczuk T, Ginalska-Malinowska M, Małunowicz EM, Grzechocińska B, Kamiński P, et al. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency - management in adults. Endokrynol Pol 2010;61:142-55.
• 6. Juul A. Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation. Int J Androl 2010;33:521-7.
• 7. Claahsen-van der Grinten HL, Hulsbergen-van de Kaa CA, Otten BJ. Ovarian adrenal rest tissue in congenital adrenal hyperplasia – a patient report. J Pediatr Endocrinol Metab 2006;19:177-82.
• 8. Stikkelbroeck NM, Hermus AR, Schouten D, Suliman HM, Jager GJ, Braat DD, et al. Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia: results of ultrasonography and MR imaging. Eur Radiol 2004;14:1802-6.
• 9. Tiosano D, Vlodavsky E, Filmar S, Weiner Z, Goldsher D, Bar-Shalom R. Ovarian adrenal rest tumor in a congenital adrenal hyperplasia patient with adrenocorticotropin hypersecretion following adrenalectomy. Horm Res Paediatr 2010;74:223-8.
• 10. Val P, Jeays-Ward K, Swain A. Identificationof a novel population of adrenal-like cells inthe mammalian testis. Dev Biol 2006;299:250-6.
• 11. King P, Paul A, Laufer E. Shh signaling regulatesadrenocortical development and identifiesprogenitors of steroidogenic lineages. Proc Natl Acad Sci USA 2009;106:21185-90.
• 12. Ketata S, Ketata H, Sahnoun A, FakhFakh H,Bahloul A, Mhiri MN. Ectopic adrenal cortex tissue: an incidental finding during inguinoscrotaloperations in pediatric patients. Urol Int 2008;81:316-9.
• 13. Dahl EV, Bahn RC. Aberrant adrenal conticaltissue near the testis in human infants. Am J Pathol 1962;40:587-98.
• 14. Jeays-Ward K, Hoyle C, Brennan J, Dandonneau M, Alldus G, Capel B, et al. Endothelial and steroidogenic cell migration are regulated by WNT4 in the developing mammalian gonad. Development 2003;130:3663-70.
• 15. Biason-Lauber A, De Filippo G, Konrad D, Scarano G, Nazzaro A, Schoenle EJ. WNT4 deficiency – a clinical phenotype distinct from the classic Mayer-Rokitansky-Kuster- Hauser syndrome: a case report. Hum Reprod 2007;22:224-9.
• 16. Mouritsen A, Jorgensen N, Main KM, Schwartz M, Juul A. Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation. Int J Androl 2010;33:521-7.
• 17. Crocker MK Barak S, Millo CM, Beall SA, Niyyati M, Chang R,et al. Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2012;97:E2084-9.
• 18. Nagamine WH, Mehta SV, Vade A. Testicular adrenal rest tumors in a patient with congenital adrenal hyperplasia: sonographic and magnetic resonance imaging findings. J Ultrasound Med 2005;24:1717-20.