Testicular adrenal rest tumors in congenital adrenal hyperplasia: a case report and literature overview

Endocr Pract. 2014 Nov;20(11):e219-24. doi: 10.4158/EP14188.CR.

Abstract

Objective: In this paper, the etiology, differential diagnosis, and therapy of testicular adrenal rest tumors (TARTs) is presented based on a patient presentation and an overview of the most recent literature concerning this subject.

Methods: Retrospective analysis of the clinical and biochemical data of an 18-year-old male patient diagnosed at the age of 2 weeks with the classic salt-wasting form of congenital adrenal hyperplasia that has been monitored in the pediatric and adolescent outpatient department since the age of 4 years.

Results: The results of adrenal hormone tests (17-hydroxyprogesterone, 17-ketosteroids, and pregnanes) had been unsatisfactory, particularly over the previous 5 years. Scrotal ultrasound detected TARTs bilaterally. After increasing the dose of hydrocortisone and introducing dexamethasone, considerable regression of the tumors was noted.

Conclusion: Lack of complete regression of the lesions is caused by fibrosis and is probably due to decreased sensitivity of adrenocorticotropic hormone and angiotensin II receptors in this tissue.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adrenal Hyperplasia, Congenital*
  • Adrenal Rest Tumor*
  • Humans
  • Hydrocortisone
  • Male
  • Retrospective Studies
  • Testicular Neoplasms*

Substances

  • Hydrocortisone