Testicular adrenal rest tumors in children with congenital adrenal hyperplasia

Saudi Med J. 2021 Sep;42(9):986-993. doi: 10.15537/smj.2021.42.9.20210257.

Abstract

Objectives: To identify testicular adrenal rest tumors (TARTs) prevalence among children with congenital adrenal hyperplasia (CAH) and to assess hormonal control role as a contributing factor for TART development. Testicular adrenal rest tumors are benign tumors complicating CAH. It affects adult males with CAH commonly, with a reported prevalence of 40% on average, and up to 94%. There is insufficient data regarding their prevalence in children aged above 4 years and adolescents.

Methods: This descriptive study included 21 children and adolescents with CAH (17 salt wasting type, 4 non salt wasting type) aged 4-20 years who were screened for TARTs by routine testicular ultrasonography from October 2012 to December 2020 at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Serum adrenocorticotropic hormone, 17-hydroxy progesterone, testosterone, dehydroepiandrosterone, and luteinizing hormone levels were measured.

Results: Testicular adrenal rest tumors were detected in 6 (28%) patients (median age: 12.5 years). The youngest affected child was 8 years old. All patients with TARTs were asymptomatic with impalpable testicular masses at the time of detection. Bilateral involvement was observed in 5 patients, while one patient had unilateral involvement. All patients with TARTs had poor hormonal control, and 4 patients had advanced bone age.

Conclusion: Testicular adrenal rest tumors development has been shown to correlate with poor hormonal control. Annual screening of young children with CAH for TART development is crucial, even for asymptomatic or with impalpable testicular masses.

Keywords: children; congenital adrenal hyperplasia; hormonal control; prevalence; testicular adrenal rest tumors.

MeSH terms

  • Adolescent
  • Adrenal Hyperplasia, Congenital* / complications
  • Adrenal Hyperplasia, Congenital* / epidemiology
  • Adrenal Rest Tumor* / complications
  • Adrenal Rest Tumor* / diagnostic imaging
  • Adrenal Rest Tumor* / epidemiology
  • Adult
  • Child
  • Child, Preschool
  • Humans
  • Male
  • Prevalence
  • Testicular Neoplasms* / complications
  • Testicular Neoplasms* / epidemiology
  • Ultrasonography