scholarly journals Sonographic Spectrum of Testicular Adrenal Rest Tumors

2018 ◽  
Vol 1 ◽  
pp. 15
Author(s):  
Zachary Nuffer ◽  
Minghao Lu ◽  
Jamal Jefferson ◽  
Vikram Dogra
Keyword(s):  
Early Diagnosis ◽  
Decision Tree ◽  
Imaging Modality ◽  
Testicular Neoplasms ◽  
Medical Attention ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

Testicular adrenal rest tumors (TARTs) are benign testicular masses but can lead to infertility without medical attention. It is important to identify TARTs in childhood, as early diagnosis has been shown to have good success in preserving fertility. It is also important to differentiate TARTs from other testicular masses to avoid unnecessary orchiectomy. Ultrasound is the preferred imaging modality for the evaluation of TARTs; however, sonographic differentiation from testicular neoplasms can sometimes be very difficult. In this article, we review the spectrum of sonographic features of TARTs and propose a decision tree that relies on these features, with the goal of increasing clinician’s confidence in diagnosing TARTs.

scholarly journals Molecular Characterization of Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia: Lesions With Both Adrenocortical and Leydig Cell Features

2015 ◽  
Vol 100 (3) ◽  
pp. E524-E530 ◽  
Author(s):  
Evelien E. J. W. Smeets ◽  
Paul N. Span ◽  
Antonius E. van Herwaarden ◽  
Ron A. Wevers ◽  
Ad R. M. M. Hermus ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Cortex ◽  
Leydig Cell ◽  
Adrenal Hyperplasia ◽  
Cell Markers ◽  
Expression Levels ◽  
Adrenal Tissue ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

Context: Testicular adrenal rest tumors (TART) are one of the major long term complications in patients with congenital adrenal hyperplasia. Although several adrenal-like properties have been assigned to these benign lesions, the etiology has not been confirmed yet. Objective: The aim of this study was to describe TART in more detail by analyzing several (steroidogenic) characteristics that may be classified as adrenal cortex or Leydig cell specific. Methods: Gene expression analysis by qPCR was performed for 14 genes in TART tissue (n = 12) and compared with the expression in healthy control fibroblasts (nonsteroidogenic control). In addition, a comparison was made with the expression levels in testis tissue (n = 9) and adrenal tissue (n = 13). Results: Nearly all genes were highly expressed in TART tissue, including all genes that encode the key steroidogenic enzymes. TART expression levels are in the majority almost identical to those found in adrenal tissue. The expression of adrenal cortex specific genes (CYP11B1, CYP11B2, and MC2R) in both TART and adrenal tissue is approximately 1000–10 000 times higher compared to that in testes samples. In addition, the Leydig cell markers INSL3 and HSD17B3 were not only found in testes, but also in TART, both at significantly higher levels than in the adrenal (p < 0.01). Conclusion: Our study shows for the first time that TART have multiple steroidogenic properties, which include not only the expression of adrenal cortex but also of Leydig cell markers. Therefore, the origin of these tumors might be a more totipotent embryonic cell type.

scholarly journals Testicular Adrenal Rest Tumors: Current Insights on Prevalence, Characteristics, Origin, and Treatment

2019 ◽  
Vol 40 (4) ◽  
pp. 973-987 ◽  
Author(s):  
Manon Engels ◽  
Paul N Span ◽  
Antonius E van Herwaarden ◽  
Fred C G J Sweep ◽  
Nike M M L Stikkelbroeck ◽  
...  
Keyword(s):  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia: 6 years of follow-up

2019 ◽  
Vol 32 (5) ◽  
pp. 519-526 ◽  
Author(s):  
Gabriela Werneck ◽  
Enda M.R. Rodrigues ◽  
Rafael M. Mantovani ◽  
Jovita S.S. Lane ◽  
Ivani N. Silva
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Tumor Progression ◽  
Repeated Measures ◽  
Laboratory Data ◽  
Hormonal Control ◽  
Adrenal Hyperplasia ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

AbstractBackgroundTesticular adrenal rest tumors (TARTs) leading to primary gonadal failure are the main etiology of infertility in congenital adrenal hyperplasia (CAH). We aimed at identifying the evolution of TART and related findings in young CAH patients.MethodsTwelve male patients (3–23 years old) with 21-hydroxilase deficiency (11 with classic salt-wasting form) were included. Testicular ultrasonography (US) was performed in two moments, by a single blinded specialist in pediatric diagnostic imaging. Tumor progression was classified according to the Response Evaluation Criteria in Solid Tumors (RECIST). The clinical and laboratory data were retrieved from medical records. Serum 17-OH-progesterone (17OHP) and androstenedione concentrations were evaluated during the whole period of follow-up, from the CAH diagnosis. A logistic regression model with repeated measures was developed for the analysis.ResultsThe prevalence of TART was 41.6% (n = 5) in the initial US evaluation and 66.6% (n = 8) after 6 years of follow-up. Tumor progression was detected in 4 of the 5 patients, and 1 presented with a stable tumor. Three patients presented with new tumors in the second evaluation. Most of the patients (n = 11) were pubertal, including a 7-year-old child with TART who presented with central precocious puberty. At regression analysis, it was observed that an inadequate hormonal control led to a 16 times greater chance of a patient to present with TART (OR = 16.08; confidence interval [CI] 95% = 2.38–108.81; p = 0.004).ConclusionsWe found a high prevalence of progressive TART in young pubertal subjects. US testicular screening should help in improving therapeutic optimization in CAH patients to reduce future impairment in fertility.

Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage

2008 ◽  
Vol 89 (3) ◽  
pp. 597-601 ◽  
Author(s):  
Hedi L. Claahsen-van der Grinten ◽  
Barto J. Otten ◽  
Ad R.M.M. Hermus ◽  
Fred C.G.J. Sweep ◽  
Christina A. Hulsbergen-van de Kaa
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Testicular Damage ◽  
Adrenal Hyperplasia ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

scholarly journals Absence of Testicular Adrenal Rest Tumors in Newborns, Infants, and Toddlers with Classical Congenital Adrenal Hyperplasia

2019 ◽  
Vol 92 (3) ◽  
pp. 157-161
Author(s):  
Mimi S. Kim ◽  
Christina M. Koppin ◽  
Pankhuri Mohan ◽  
Fariba Goodarzian ◽  
Heather M. Ross ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Infants And Toddlers ◽  
Adrenal Hyperplasia ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
Testicular Adrenal Rest

scholarly journals Testicular adrenal rest tumors in boys with 21-hydroxylase deficiency, timely diagnosis and follow-up

2018 ◽  
Vol 7 (4) ◽  
pp. 544-552 ◽  
Author(s):  
Mirjana Kocova ◽  
Vesna Janevska ◽  
Violeta Anastasovska
Keyword(s):  
Metabolic Control ◽  
Ultrasound Examination ◽  
Genetic Mutations ◽  
Ultrasound Screening ◽  
Hydroxylase Deficiency ◽  
Timely Diagnosis ◽  
Testicular Adrenal Rest Tumors ◽  
Adrenal Rest ◽  
21 Hydroxylase Deficiency ◽  
Testicular Adrenal Rest

Background Testicular adrenal rest tumors (TARTs) are found in 30–94% of adult males with congenital adrenal hyperplasia (CAH). We sought to explore TART appearance through yearly ultrasound examination of testes in young boys with CAH, and its association with metabolic control and genetic mutations. Methods Twenty-five boys with 21-hydroxylase deficiency in the age group 4–18 years diagnosed during the period 2001–2016 were included in the study. ACTH, 17-hydroxyprogesterone, androstenedione and testosterone were measured at 4-month intervals. Growth and BMI were assessed at the time of evaluation. PCR/ACRS method was used for CYP21A2 gene analysis. Testicular ultrasound examination was performed yearly. Results TARTs were detected by ultrasound in 8 children at the age of 6–16 years (13.2 years average). Five had salt-wasting form, two had simple virilizing form and one had non-classic form of CAH. Significant differences in the17OHP and androstenedione levels were detected between the boys, adherent and non-adherent to therapy. Inadequate metabolic control was not different in boys with and without TART (11/17 and 5/8 respectively). No significant difference was detected in the distribution of genetic mutations or adherence to therapy between patients with and without TARTs. One patient had a mutation not reported thus far in TART and another developed leukemia. Conclusion TART is not rare in young boys with CAH, irrespective of the specific mutation or metabolic control. Ultrasound screening helps timely diagnosis and adjustment of therapy.

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