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

Dominika Januś, Małgorzata Wójcik, Katarzyna Tyrawa, Karolina Popławska, Jerzy B Starzyk

Journal: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2019;20(11):e219-24

PMID: 25100387

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.

Address: Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, Jagiellonian University Medical College, Krakow, Poland.; Department of Pediatrics, Chair of Pediatrics, Jagiellonian University Medical College, Krakow, Poland.
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