Testicular Adrenal Rest Tumors: a rare (but typical) case report
Number:27ESURABS0045
Type:Scientific Electronic Presentation
Authors: Carolina Tim-Tim, Margarida Lima, João Pedro Caldeira, Mónica Coutinho
Keywords:Adrenal rest tumors; testicular neoplasms; congenital adrenal hyperplasia; ultrasound
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Objectives

Testicular adrenal rest tumors (TARTs) are a rare cause of benign testicular masses that frequently present in a distinctive clinical setting.

With this poster, we aim to present a case report of a patient with the diagnosis of congenital adrenal hyperplasia (CAH) and bilateral TARTs, focusing on the typical clinical presentation, differential diagnosis and the most frequent imaging features on ultrasound.


Material and methods

We present the case of a twenty-five-year-old male patient, diagnosed with CAH since the neonatal period, that started follow-up in our institution because of testicular nodules found on physical examination.

The patient had no remarkable medical history besides the diagnosis of pituitary adenoma with features of acromegaly treated with hypophysectomy.

Testicular ultrasound showed bilateral multiple confluent nodules, with heterogeneous echostructure, predominantly hypoechoic, showing signs of hypervascularity on color doppler. These imaging findings, together with the known diagnosis of CAH made the hypothesis of TARTs very likely, and sonographic follow-up was the chosen approach.

Despite treatment with corticosteroids, the patient showed signs of poor control of the disease that reflected on high levels of adrenocorticotropic hormone (ACTH),...

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Sagittal and transverse view of right and left testis showing the typical imaging features of testicular adrenal rest tumors on ultrasound seen in our patient. Ultrasound shows testicular contour lobulation, associated with bilateral, multiple, and confluent nodules, with heterogenous echogenicity, predominantly hypoechoic. The nodules occupy the mediastinum testis, as well as most of the surrounding testicular tissue.
Sagittal views comparing the imaging findings in the right testis with and without eco-Doppler. Color Doppler images illustrate the hypervascularity of the nodules that is frequent in testicular adrenal rest tumors.

Results

TARTs are a rare cause of benign testicular masses that mostly occur in patients with CAH.

CAH is a group of autosomal recessive disorders affecting steroid synthesis of the adrenal cortex. They are caused by a defect of an enzyme involved in adrenal steroid production, most commonly 21-hydroxylase deficiency. There is a continuum of disease severity in this group of disorders, which depends on the residual enzyme activity, with different steroid synthesis impairment, all leading to high levels of ACTH.

The etiopathogenesis of TARTs is not completely understood and is still a topic of debate. Because of their resemblance with adrenal tissue, these lesions were originally called “testicular adrenal rest tumors”. Recent studies found that cells that originate the tumors have both testicular and adrenal characteristics, which indicates a pluripotent cell type...

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Table 1: Differences and similarities between TARTs and LCTs

Conclusions

TARTs are a rare entity that must be included in the differential diagnosis of testicular masses in the appropriate clinical setting, such as male patients with CAH, so that an unnecessary orchiectomy can be avoided.

These tumors have their origin on pluripotent cells with adrenal and testicular characteristics, that proliferate in the presence of high levels of ACTH. They are not malignant but longstanding growth can result in irreversible damage of testicular tissue. They represent the most frequent cause of subfertility in male patients with CAH.

The reported clinical case illustrates the typical clinical presentation and imaging features of a patient with CAH and bilateral TARTs tumors, with evolution to testicular function impairment.

Ultrasound is the imaging modality of choice for diagnosis. TARTs usually present as bilateral, multiple, confluent...

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