Original articles

Ultrasonographic characteristics of testicular adrenal residue tumor in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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  • Department of Ultrasound, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China

Received date: 2021-10-19

  Online published: 2023-01-29

Abstract

Objective: To summarize the ultrasonographic characteristics of testicular adrenal tumor (TART) in male patients with congenital adrenocortical hyperplasia (CAH) due to 21-hydroxylase deficiency, and to improve the understanding of sonographers on TART. Methods: Twenty-one CAH patients due to 21-hydroxylase deficiency were collected. All patients were found to have abnormal echoes in the testis by scrotal high-frequency ultrasound examination and were diagnosed as TART. The ultrasonic images of TART lesions were observed, and the number and growth mode of the lesions involving the testis were evaluated. The morphology, boundary, echogenicity and blood supply characteristics, and epididymis involvement were observed. Results: Among the 21 patients, 14 patients had bilateral testicular lesions with the same ultrasound features, and seven patients were unilateral. Two patients were epididymal involvement. Among the total 35 testicular lesions, 82.9% (29/35) grew along the testicular mediastinum, 17.1% (6/35) grew diffusely. Hypoechoic lesions accounted for 51.4% (18/35), heterogeneous hyperechoic lesions accounted for 28.6% (10/35), and heterogeneous hyperechoic lesions with coarse calcification accounted for 20%(7/35). The lesion morphology mainly showed irregular lobular shape and round shape, accounting for 85.7% (30/35) and 14.3% (7/35), respectively. The rich blood supply lesions accounted for 62.8% (22/35), and 22.9% (8/35) and 14.3% (5/35) of the lesions were hypovascular or anvascular. Conclusions: Sonographic appearance of TART is variable but still characteristic. The lesions present as bilateral testis with rich blood supply and irregular hypoechoic growth along the mediastinum. The ultrasonic features of the bilateral lesions are the same. With the history of CAH, it might provide clues for diagnosis of TART.

Cite this article

WANG Zhiqian, LI Min, YÜ Yifei, ZHOU Jianqiao . Ultrasonographic characteristics of testicular adrenal residue tumor in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency[J]. Journal of Diagnostics Concepts & Practice, 2022 , 21(05) : 588 -591 . DOI: 10.16150/j.1671-2870.2022.05.007

References

[1] Corcioni B, Renzulli M, Marasco G, et al. Prevalence and ultrasound patterns of testicular adrenal rest tumors in adults with congenital adrenal hyperplasia[J]. Transl Androl Urol, 2021, 10(2):562-573.
[2] Witchel SF. Non-classic congenital adrenal hyperplasia[J]. Steroids, 2013, 78(8):747-750.
[3] Speiser PW, Arlt W, Auchus RJ, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an endocrine society clinical practice guideline[J]. J Clin Endocrinol Metab, 2018, 103(11):4043-4088.
[4] Arlt W, Willis DS, Wild SH, et al. Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients[J]. J Clin Endocrinol Metab, 2010, 95(11):5110-5121.
[5] Delfino M, Elia J, Imbrogno N, et al. Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia: prevalence and sonographic, hormonal, and seminal characteristics[J]. J Ultrasound Med, 2012, 31(3):383-388.
[6] Wang Z, Yang Z, Wang W, et al. Diagnosis of testicular adrenal rest tumors on ultrasound: a retrospective study of 15 cases report[J]. Medicine (Baltimore), 2015, 94(36):e1471.
[7] Deshpande Sss, Shetty D, Saifi S. Sonographic appearance of testicular adrenal rest tumour in a patient with con-genital adrenal hyperplasia[J]. Pol J Radiol, 2017, 82:526-529.
[8] Sharbidre KG, Lockhart ME. Imaging of scrotal masses[J]. Abdom Radiol (NY), 2020, 45(7):2087-2108.
[9] Shahab N, Doll DC. Testicular lymphoma[J]. Semin Oncol, 1999, 26(3):259-269.
[10] Crellin AM, Hudson BV, Bennett MH, et al. Non-Hodgkin' s lymphoma of the testis[J]. Radiother Oncol, 1993, 27(2):99-106.
[11] Aggarwal N, Parwani AV. Spermatocytic seminoma[J]. Arch Pathol Lab Med, 2009, 133(12): 1985-1988.
[12] Finkielstain GP, Kim MS, Sinaii N, et al. Clinical char-acteristics of a cohort of 244 patients with congenital a-drenal hyperplasia[J]. J Clin Endocrinol Metab, 2012, 97(12):4429-4438.
[13] Mikuz G. Update on the pathology of testicular tumors[J]. Anal Quant Cytopathol Histpathol, 2015, 37(1):75-85.
[14] Claahsen-van der Grinten HL, Stikkelbroeck N, Falham-mar H, et al. Managemnt of endocrine disease: gonadal dysfunction congenital adrenal hyperplasia[J]. Eur J Endocrinol, 2021, 184(3):R85-R97.
[15] Ma L, Xia Y, Wang L, et al. Sonographic features of the testicular adrenal rests tumors in patients with congenital adrenal hyperplasia: a single-center experience and litera-ture review[J]. Orphanet J Rare Dis, 2019, 14(1):242.
[16] Poyrazoglu S, Saka N, Agayev A, et al. Prevalence of testicular microlithiasis in males with congenital adrenal hyperplasia and its association with testicular adrenal rest tumors[J]. Horm Res Paediatr, 2010, 73(6):443-448.
[17] Reisch N, Rottenkolber M, Greifenstein A, et al. Testicu-lar adrenal rest tumors develop independently of long-term disease control: a longitudinal analysis of 50 adult men with congenital adrenal hyperplasia due to classic 21-hydroxylase deficiency[J]. J Clin Endocrinol Metab, 2013, 98(11):E1820-E1826.
[18] Merke DP, Auchus RJ. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency[J]. N Engl J Med, 2020, 383(13):1248-1261.
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