Uropathology

2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

Congenital Adrenal Hyperplasia in the Testis

  • Manuel NistalEmail author
  • Pilar González-Peramato
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4794
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Synonyms

Adrenogenital syndrome-associated tumors; Testicular adrenal rest tumors (TARTs); Testicular adrenogenital tumors; Testicular tumors in congenital adrenal hyperplasia; Testicular tumors of the adrenogenital syndrome (TTAGS)

Definition

Testicular adrenal rest tumors (TARTs) are nodular formations, macro or microscopic, benign, located in the vicinity of the rete testis, constituted by cells similar to those of the adrenal cortex. They develop in patients with congenital adrenal hyperplasia (CAH) who have followed inappropriate treatment.

Clinical Features

Patients with TARTs are carriers of a CAH, an autosomal recessive process characterized by deficit of enzymes involved in the synthesis of glucocorticoids. The deficit of corticoids causes an increase in ACTH secretion, resulting in adrenal hyperplasia. The stimulation of ectopic adrenal tissue in the testicle or the stimulation of pluripotent cells of the testicular hilum leads to the development of TARTs. In children, they...
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References and Further Reading

  1. Deshpande, S. S., Shetty, D., & Saifi, S. (2017). Sonographic Appearance of Testicular Adrenal Rest Tumour in a Patient with Congenital Adrenal Hyperplasia. Pol J Radiol, 82, 526–529.Google Scholar
  2. Kocova, M., Janevska, V., & Anastasovska, V. (2018). Testicular adrenal rest tumors in boys with 21-hydroxylase deficiency, timely diagnosis and follow-up. Endocr Connect, 7, 544–552.Google Scholar
  3. Lottrup, G., Nielsen, J. E., Skakkebæk, N. E., Juulm, A., & Rajpert-De Meyts, E. (2015). Abundance of DLK1, differential expression of CYP11B1, CYP21A2 and MC2R, and lack of INSL3 distinguish testicular adrenal rest tumours from Leydig cell tumours. European Journal of Endocrinology, 172, 491–499.PubMedCrossRefGoogle Scholar
  4. Nistal, M., Gonzalez-Peramato, P., & Serrano, A. (2017). Differential diagnosis of tumors in the adrenogenital syndrome. In Clues in the diagnosis of non-tumoral testicular pathology (pp. 51–57). Cham: Springer.CrossRefGoogle Scholar
  5. Reisch, N., Flade, L., Scherr, M., Rottenkolber, M., Pedrosa Gil, F., Bidlingmaier, M., Wolff, H., Schwarz, H. P., Quinkler, M., Beuschlein, F., & Reincke, M. (2009). High prevalence of reduced fecundity in men with congenital adrenal hyperplasia. The Journal of Clinical Endocrinology and Metabolism, 94, 1665–1670.PubMedCrossRefGoogle Scholar
  6. Rutgers, J. L., Young, R. H., & Scully, R. E. (1988). The testicular “tumor” of the adrenogenital syndrome. A report of six cases and review of the literature on testicular masses in patients with adrenocortical disorders. The American Journal of Surgical Pathology, 12, 503–513.PubMedCrossRefGoogle Scholar
  7. Tanaka, M., Enatsu, N., Chiba, K., & Fujisawa, M. (2017). Two cases of reversible male infertility due to congenital adrenal hyperplasia combined with testicular adrenal rest tumor. Reprod Med Biol, 17, 93–97.Google Scholar
  8. Wang, Z., Yang, S., Shi, H., Du, H., Xue, L., Wang, L., Dong, Y., & Han, A. (2011). Histopathological and immunophenotypic features of testicular tumour of the adrenogenital syndrome. Histopathology, 58, 1013–1018.CrossRefPubMedGoogle Scholar
  9. Yu, M. K., Jung, M. K., Kim, K. E., Kwon, A. R., Chae, H. W., Kim, D. H., & Kim, H. S. (2015). Clinical manifestations of testicular adrenal rest tumor in males with congenital adrenal hyperplasia. Ann Pediatr Endocrinol Metab, 20, 155–161.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Anatomy, Histology and Neuroscience, School of MedicineUniversidad Autónoma de MadridMadridSpain
  2. 2.Department of PathologyUniversidad Autónoma de MadridMadridSpain
  3. 3.Department of PathologyUniversity Hospital La PazMadridSpain