The prevalence of testicular adrenal rest tumours varies in different forms of congenital adrenal hyperplasia. Patients with 21-hydroxilase deficiency usually have bilateral and palpable testicular nodules. Although adrenal rest tumours are well documented in the literature, the diagnosis and management require a multidisciplinary approach: the cooperative work of endocrinologists, urologists, pathologists and radiologists is essential. In the case of an early diagnosis, appropriately increased corticosteroid treatment may reduce the tumour mass. In advanced stages, tumours can lead to irreversible parenchymal damage causing infertility. The importance of an early and accurate diagnosis cannot be emphasized enough, since the therapy differs significantly from other benign or malignant testicular neoplasia. A case of a testicular adrenal rest tumour is presented along with the multidisciplinary perspectives of the diagnosis and management of these lesions. Orv Hetil. 2020; 161(16): 623–631.

https://akjournals.com/downloadpdf/journals/650/161/16/article-p623.pdf

Testicular adrenal rest tumor in the background of congenital adrenal hyperplasia

Congenital adrenal hyperplasia (CAH) refers to a group of autonomic disorders due to enzyme deficiency for the biosynthesis of steroid hormones. These disorders entail an increase in ACTH levels and as followed by adrenal hyperplasia. CAH is categorized into two types, classic and non-classic. In the common type we have a deficiency of 21-hydroxylase observed in a prevalence of 1 per 5000 per 145,000 births. We have insufficient aldosterone and cortisol production and as a result, elevated plasma ACTH levels, with subsequent disorders that this entails, depending on the level of deficiency. Testicular adrenal rest tumor (TART) develops from isletsectopic adrenal tissue within the gonads, directly affected by ACTH overproductions a complication of CAH, with a prevalence ranging from 27% to 47%. These are benign tumors that are recognized as palpable masses. A biopsy of these tumors is recommended as well as their surgical removal, so as to rule out malignancy.In this case report we present a case of bilateral synchronous TART tumor in association with medullolipoma in a patient with CAH. The dilemma arises regarding the decision of bilateral orchiectomy.

Bilateral Synchronous Testicular Mass from Testicular Adrenal Rest Tumors, in a Patient with Congenital Adrenal Hyperplasia, or Testicular Leydig Cell Tumors? Dilemma for Bilateral Orchiectomy or Not

21-hydroxylase deficiency (21-OHD) caused congenital adrenal hyperplasia (CAH) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO) synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs) are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The patient showed physical signs of precocious puberty. The levels of blood adrenocorticotropic hormone (ACTH), urinary 17-ketone steroids (17-KS), dehydroepiandrosterone sulfate (DHEA-S), and serum progesterone (PRGE) were elevated, whereas those of follicle-stimulating hormone (FSH), luteinizing hormone (LH), and CO were reduced. Computed tomography (CT) of the adrenal glands and magnetic resonance imaging (MRI) of the testes showed a soft tissue density (more pronounced on the right side) and an irregularly swollen mass (more pronounced on the left side), respectively. Pathological examination of a specimen of the mass indicated polygonal/circular eosinophilic cytoplasm, cord-like arrangement of interstitial cells, and lipid pigment in the cytoplasm. Immunohistochemistry results precluded a diagnosis of Leydig cell tumors. DNA sequencing revealed a hackneyed homozygous mutation, I2g, on intron 2 of the CYP21A2 gene. The patient's symptoms improved after a three-month of dexamethasone therapy. Recent radiographic data showed reduced hyperplastic adrenal nodules and testicular tumors. A diagnosis of TART should be considered and prioritized in CAH patients with testicular tumors. Replacement therapy using a sufficient amount of dexamethasone in this case helps combat TART.

https://www.ijfs.ir/article_45351_2ae9cdfab26e2d6ab354a12ef1209b44.pdf

A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

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A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.