Male factorTesticular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage
Section snippets
Patients
Seven male patients with CAH caused by 21-hydroxylase deficiency and longstanding, bilateral TART were treated with testis-sparing surgery because of infertility (n = 5), poor medical control (n = 2), and testicular pain and discomfort (n = 2). The age of the patients was 30 ± 8.9 years (mean ± SD; range, 23–51 y). Four of these patients (patients 3, 5, 6, and 7) had been treated with high doses of glucocorticoids to reduce tumor size, without success. All but one patient had the classic
Testicular Tumors
Tumor weight and histological characterization are listed in Table 1. Weight of the tumors in the left testes was 9.8 ± 9.9 g (mean ± SD; range, 1.3–27.8 g) and in the right testes, it was 8.7 ± 9.1 g (range, 0.45–22.9 g). Macroscopically, all tumors were firm and multilobular, with a yellow to tan color on cut surface and narrow bands of fibrous tissue. The maximal diameter of the tumors varied from 1 to 7 cm.
On microscopy, the tumors were sharply demarcated but unencapsulated and consisted of
Discussion
The occurrence of TART is an important complication in male patients with CAH. The tumors have no malignant features, and therefore, there appears to be no need to remove them at an early stage. However, because of the central localization of the tumors near the mediastinum testis, compression of the seminiferous tubules may lead to obstructive azoospermia and irreversible damage of the testes. We found a decreased tubular diameter and a varying degree of peritubular fibrosis and tubular
Acknowledgments
The authors acknowledge Eva Morava, M.D., and Maria Cash for critical reading of the manuscript.
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2023, Genetic Steroid Disorders: Second EditionCongenital adrenal hyperplasia owing to 21-hydroxylase deficiency
2023, Genetic Steroid Disorders: Second EditionGenotype of congenital adrenal hyperplasia patients with testicular adrenal rest tumor
2022, European Journal of Medical GeneticsCitation Excerpt :A study reported that the incidence of TART increases up to 94% in adults (Stikkelbroeck et al., 2001). A few studies evaluated the incidence of TART in childhood&adolescence, and some of them included pediatric and adult age groups (Avila et al., 1996; Vanzulli et al., 1992; Claahsen-van der Grinten et al., 2008; Claahsen-van der Grinten et al., 2007a; Cakir et al., 2012; Claahsen-van der Grinten et al., 2007b). It is reported that the prevalence of TART in children is above 24% (Claahsen-van der Grinten et al., 2007b).
Adrenal disorders
2021, Biochemical and Molecular Basis of Pediatric DiseaseThree Cases of Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia—A Diagnostic and Therapeutic Challenge
2019, UrologyCitation Excerpt :In the other patients, daily glucocorticoid doses of 0.25 mg dexamethasone and 15 mg hydrocortisone (case 2) and 0.5 mg dexamethasone additional to 15 mg hydrocortisone (case 3) were chosen and proved to be effective concerning patients’ endocrine disease control. TARTs are not malignant but can do severe and even irreversible testicular damage by compression of the seminiferous tubules and toxic paracrine effects.16 Thus, semen analysis should be performed to evaluate fertility.