Sonographic features of the testicular adrenal rests tumors in patients with congenital adrenal hyperplasia: a single-center experience and literature review

Abstract

Purpose: Testicular adrenal rests tumor (TART) is a rare kind of benign tumor in the testis. It usually occurred secondary to congenital adrenal hyperplasia (CAH), a hormonal disorder caused by hydroxylase deficiency. As the first-line examination method, ultrasound provides crucial diagnostic information for TART, although misdiagnosis to malignancy is quite common because of its rare prevalence. We aimed to summarize the sonographic manifestations of TART to improve the diagnostic accuracy and specificity. Methods: Eight CAH patients with TART identified by ultrasound in our medical center were retrospectively reviewed. Clinical and hormonal profile, semen analysis and treatment choices were collected. Sonographic examinations were performed at the first evaluation and interpreted by experienced radiologists individually. All patients received regular follow-up, and 5 patients undertook repeated scrotal ultrasound. A literature review of TART in CAH patients was conducted, with 123 patients from 23 articles since 1990 included. Results: A total of 8 patients aged between 4 to 27 years old were enrolled. 7 of 8 (87.5%) patients exhibited bilateral testicular lesions. The sizes of the testicular lesions were between 0.18 ml to 5.68 ml, and all showed a clear boundary. 10/15 (66.7%) lesions were homogenously hypoechoic, 4/15 (26.7%) were heterogeneously isohypoechoic, and 1/15 (6.7%) were homogenously isoechoic. 10/15 (66.7%) lesions were hyper-vascular. The longitudinal follow-up of 5 patients showed testicular lesions changed in terms of size, echogenicity, and vascularity after steroid treatment. A potential correlation may exist between ACTH levels and tumor size (p = 0.066). From the literature review, 100/123 (81%) patients got bilateral lesions, and 95% of them were located near the mediastinum. 80/103 (78%) lesions exhibited a clear boundary, and predominant lesions (74%) were hypoechogenic. Vascularity was with great diversity. Seventy-nine lesions of 44 patients were followed-up by scrotal ultrasound, among which 29 (37%) remained unchanged, 29(37%) shrank, and 21(27%) disappeared. Conclusions: Key sonographic characteristics of TART are: resembled lesions on both testes, located near the mediastinum, clear boundary, and changed in size or echogenicity after steroid treatment. These features can help radiologists to make an accurate diagnosis of TART.

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

“…In surgical pathology, most adrenal rests are discovered as incidental microscopic findings in various surgical specimens including kidneys or gonads (particularly, spermatic cord [22], rete testis [23][24][25], epididymis [26], ovary/paraovarian [22,[27][28][29][30][31][32][33], salpinx and parasalpinx [22,34,35] ). A recent surgical series from adults identified ectopic adrenal rests in both males and females with a more frequent Fig.…”

Congenital adrenal hyperplasia with associated giant adrenal myelolipoma, testicular adrenal rest tumors and primary pigmented nodular adrenocortical disease: A case report and brief review of the literature

“…The main complication associated with TART is infertility as a result of testicular structural and blood flow impairment secondary to tumor burden [9] . Exogenous steroid treatment may provide mass stability or reduce TART's mass burden and reverse infertility [13] . The main differential diagnosis to consider for these lesions are Leydig cell tumors which be malignant and are difficult to distinguish from TART's histologically.…”

Case Report:clinical experience of bilateral giant pediatric Testicular adrenal rest tumors with 3 Beta-Hydroxysteroid Dehydrogenase-2 family history

“…In fact,most TARTs location is in the middle site of testis [7,8]that is undetectable in physical examination and that is why we recommend ultrasound as the first means to screen testis tumor. Most TARTs are hypoechoic and bilateral while vascularity remains controversial for lack of objective values,machine settings difference and subjective judgment [9,10]. In Magnetic Resonance Imaging(MRI), TARTs characterized with hypointensity on T2-W images, and hyperintensity or iso-intensity on T1-W images and lesion margins were clearly defined [5].Owing to overlapping image results in Leydig tumors, clinical information and laboratory testing should be combined correct diagnosis.In fact, Miroslav Dumic [11] found that TARTs patients have been in stage 3 or 4 which should have accepted the early ultrasound examination.…”