Case Report

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

Volume: 6 Number: 1 January 4, 2020
EN

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

Abstract

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. 

Keywords

References

  1. 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. 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. 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. 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. 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. 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. 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. 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.

Details

Primary Language

English

Subjects

Endocrinology

Journal Section

Case Report

Publication Date

January 4, 2020

Submission Date

September 10, 2018

Acceptance Date

February 24, 2019

Published in Issue

Year 2020 Volume: 6 Number: 1

AMA
1.Aydın Buyruk B, Yorulmaz G, Değer AN, Kebapçı MN. Congenital adrenal hyperplasia with an ovarian adrenal rest tumor: a case report. Eur Res J. 2020;6(1):77-82. doi:10.18621/eurj.435638