The Role of MRI Findings in the Treatment of Testicular Adrenal Rest Tumors in a Child With Salt-Wasting Congenital Adrenal Hyperplasia: A Case Report

TL;DR

A child with adrenal hyperplasia developed testicular tumors due to poor treatment, diagnosed and monitored using MRI.

Contribution

Highlights the importance of MRI in diagnosing and monitoring TARTs in pediatric CAH patients with poor hormonal control.

Findings

MRI revealed bilateral TARTs in a child with poorly controlled CAH.

TARTs showed T2-hyperintense features with mild contrast enhancement on MRI.

Early MRI detection is crucial for managing TARTs and preserving reproductive function.

Abstract

Testicular adrenal rest tumors (TARTs) are benign, adrenal-like hyperplastic lesions that occur ectopically within the testes. They are most commonly observed in male patients with congenital adrenal hyperplasia (CAH), especially in those with inadequate glucocorticoid therapy and consequent poor hormonal control. Chronic elevation of adrenocorticotropic hormone (ACTH), driven by insufficient cortisol replacement, stimulates the growth of adrenal rest cells within the testes, leading to tumor formation. Although TARTs are non-malignant, their clinical significance lies in the potential for testicular dysfunction, structural abnormalities, and infertility if left undiagnosed or untreated. Early detection through imaging is essential to guide clinical management and preserve reproductive function.

We report the case of an 11-year-9-month-old boy diagnosed with the salt-wasting form of CAH secondary to 21-hydroxylase deficiency. Due to long-standing poor compliance with glucocorticoid therapy, the patient developed bilateral TARTs as a result of chronic ACTH overstimulation. Magnetic resonance imaging (MRI) of the scrotum revealed multiple well-defined, bilateral testicular masses that appeared T2-hyperintense with mild post-contrast enhancement, features consistent with TARTs.

This case emphasizes the pivotal role of MRI in the accurate diagnosis and longitudinal monitoring of TARTs in pediatric patients with CAH, particularly those with suboptimal hormonal control.

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