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

Department of Pathology, Harvard Medical School, Boston, Massachusetts.
American Journal of Surgical Pathology (Impact Factor: 5.15). 08/1988; 12(7):503-13.
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The clinical and pathological features of 40 cases in which testicular masses developed in patients with the adrenogenital syndrome are reviewed; this study was based on six personally observed cases and 34 other cases in the literature. The adrenal disorder was of the salt-losing form in two-thirds of the cases and the non-salt-losing form in the other third. Although the clinical diagnosis of the adrenogenital syndrome had been established prior to the discovery of the testicular lesion in most of the patients, in 18% of them the diagnosis was not made until or after the development of a testicular mass. Two-thirds of the masses were palpable (up to 10 cm); these cases were usually discovered in early adult life (average, 22.5 years). The remaining one-third were small (under 2 cm) and were usually found in children either at autopsy or on testicular biopsy. Eighty-three percent of the masses were bilateral. Eighty-six percent of the small lesions were located in the hilus. The larger lesions involved the testicular parenchyma in all but one case. They formed well-demarcated but unencapsulated brown-green masses, typically separated into lobules by prominent bands of fibrous tissue. Microscopical examination revealed sheets, nests, and (rarely) cords of cells with abundant eosinophilic cytoplasm separated by bands of fibrous tissue. Lipochrome pigment was identified in the cytoplasm in many cases, but crystals of Reinke were uniformly absent. The major pathological differential diagnosis is Leydig cell tumor; the associated clinical and laboratory features--including the high frequency of bilaterality and a decrease in the size of the tumor with corticosteroid therapy--are diagnostic of a testicular "tumor" of the adrenogenital syndrome. Although a variety of origins have been suggested for these lesions, in our opinion an origin from hilar pluripotential cells, which proliferate as a result of the elevated level of adrenocorticotropic hormone, is most likely.

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    • "It has been suggested that adrenal rest tumors consist of adrenal tissues localized in the scrotum, within the testicles (7). In patients with CAH, TART are usually bilaterally localized, and tumor size decreases with adequate steroid replacement therapy (8). It has been reported that these tumors cause infertility in adult life via destruction of the normal testes in patients with CAH (9). "
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    ABSTRACT: Early diagnosis and treatment of testicular adrenal rest tumors (TART) is important for gonadal functions and fertility protection in boys with congenital adrenal hyperplasia (CAH). In this descriptive study, we investigated the prevalence of TART in boys with 21-hydroxylase deficient (21OHD) CAH followed in our pediatric endocrine clinic. The study group consisted of 14 male patients with a mean age of 9.6 ± 5.1 (range: 0.8-18.3) years. Six (42.9%) of the 14 patients were diagnosed as having salt-wasting type (SW) and eight (57.1%) patients - as having the simple virilizing (SV) form of 21OHD. Mean age at diagnosis was 2.9 ± 2.7 (range: 0.03-6.3) years. Two different radiologists performed scrotal ultrasonography. Chronological age, bone age, and anthropometric measurements were evaluated. Serum adrenocorticotropic hormone (ACTH), 17-alpha-hydroxyprogesterone (17OHP) and androstenedione levels were also evaluated in all patients during the follow-up period. Scrotal ultrasonography revealed bilateral TART in two patients (14.3%) and testicular microlithiasis (TM) in four patients (28.6%). One patient had both TART and TM bilaterally. During the follow-up period, the mean serum adrenocorticotropic hormone, 17OHP and androstenedione levels in the total group of patients were 130.0 ± 179.1 pg/mL (21.7-726.5), 5.8 ± 3.3 ng/mL (0.8-11.4) and 4.3 ± 4.1 (0.2-11.0) ng/mL, respectively. Microlithiasis or TART may be frequently encountered during the follow-up of patients with CAH. In order to prevent late complications including infertility, we suggest that ultrasonographic evaluations be performed yearly in all male CAH patients.
    Journal of Clinical Research in Pediatric Endocrinology 06/2012; 4(2):94-100. DOI:10.4274/jcrpe.563
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    • "With a BMI within the range of overweight [21], obesity in the CAH patients, and especially the higher risk of obesity in CAH children as compared to the background population [22], may further aggravate a hyperandrogenic condition. Another explanation for larger ovarian volumes in the CAH patients may be ovarian adrenal rest tumours, which may originate from pluripotent cells [23]. As the adrenals develop in the immediate vicinity of the gonads, some adrenal tissue may adhere to the gonads, and thereby descent with the testis or the ovary [24]. "
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    ABSTRACT: Background. Our knowledge on long-term outcome in CAH remains incomplete. Methods. In a prospective study (33 CAH patients, 33 age-matched controls), reproductive outcomes, self-rating of genital appearance and function, and sexuality were correlated to degree of initial virilisation, genotype, and surgery. Results. Patients had larger median clitoral lengths (10.0 mm [range 2–30] versus 3.5 [2–8], P < .001), shorter vaginal length (121 mm [100–155] versus 128 [112–153], P = .12), lower uterine volumes (29.1 ml [7.5–56.7] versus 47.4 [15.9–177.5], P = .009), and higher ovarian volumes (4.4 ml [1.3–10.8] versus 2.8 [0.6–10.8], P = .09) than controls. Satisfaction with genital appearance was lower and negatively correlated to degree of initial virilisation (rs = ≤−0.39, P ≤ .05). More patients had never had intercourse (P = .001), and age at 1st intercourse was higher (18 yrs versus 16 yrs, P = .02). Conclusion. Despite overall acceptable cosmetic results, reproductive outcomes were suboptimal, supporting that multidisciplinary teams should be involved in adult follow up of CAH patients.
    International Journal of Pediatric Endocrinology 10/2010; 2010(2):784297. DOI:10.1155/2010/784297
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    • "These tumours were first reported in 1940 by Wilkins et al. [4]. Since then testicular tumours have been described in several papers, mainly case reports [5] [6] [7] [8] [9]. Because of the morphological and functional resemblance with adrenal tissue they are called " testicular adrenal rest tumours " (TART). "
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    ABSTRACT: In adult patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumours (TART) is an important complication leading to gonadal dysfunction and infertility. These tumours can be already found in childhood and puberty. In this paper, we review the embryological, histological, biochemical, and clinical features of TART and discuss treatment options.
    International Journal of Pediatric Endocrinology 02/2009; 2009(1):624823. DOI:10.1155/2009/624823
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