Testicular adrenal rest tumours in congenital adrenal hyperplasia

Department of Paediatric Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands.
Best Practice & Research: Clinical Endocrinology & Metabolism (Impact Factor: 4.91). 05/2009; 23(2):209-20. DOI: 10.1016/j.beem.2008.09.007
Source: PubMed

ABSTRACT In adult patients with congenital adrenal hyperplasia (CAH) the presence of testicular adrenal rest tumours (TART) is an important cause of gonadal dysfunction and infertility. In the last decade several papers have focused on the origin and pathogenesis of these tumours. In this paper we review the embryological, histological, biochemical and clinical features of TART and discuss the treatment options. Furthermore, we propose a new five-stage classification of TART, based on sonographic, clinical and biochemical parameters, that may lead to a better follow up and treatment of patients with TART.

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    • "They may sometimes significantly enlarge, mimicking testicular malignant tumors and leading to damage to the structure of testes, spermatogenesis disorders and infertility [1]. Until now, the diagnostics of TART have been based on a combination of clinical features (syndromes with hypercorticotropinemia), imaging methods (primarily two dimensional ultrasound e 2D US), response of the foci to glycocorticosteroid (GCS) therapy and exclusion of the neoplastic process [1] [2]. Although numerous investigations have been conducted, a specific marker useful in the diagnosis and precise identification of the nature of the TART tissue is still unknown. "
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    ABSTRACT: BACKGROUND: Testicular adrenal rest tumors (TART) are the nodular testicular lesions deriving from the adrenal remnant tissue reported in boys and men with congenital adrenal hyperplasia. Until now, the diagnostics of TART have been based on a combination of clinical features, imaging methods (primarily two dimensional ultrasound - 2D US), response of the foci to glycocorticosteroid (GCS) therapy and exclusion of the neoplastic process. Application of 2D US supplies however a limited range of information about the volume, demarcation, structure and vascularization of the lesions. OBJECTIVE: To define whether the use of 3D US, power Doppler and elastography changes the algorithm of the diagnostics and monitoring or treatment of TART. MATERIAL AND METHODS: In this study, modern ultrasound techniques such as 3D US and elastography were introduced in two boys with TART. RESULTS: The 3D power Doppler option gives the opportunity for accurate assessment of the volume of testes and adrenal tissue foci and their vascularization. Sonographic elastography allows the assessment of stiffness of adrenal tissue areas compared to normal testis parenchyma. CONCLUSION: The use of these modern techniques enables more adequate and advanced diagnostics, and more precise monitoring of the effects of treatment in patients with TART.
    Journal of pediatric urology 03/2013; 9(6). DOI:10.1016/j.jpurol.2013.02.008 · 1.41 Impact Factor
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    • "These reports have raised concerns about the distinction of LCT from TARTs. Such distinction is interesting, given that there is a different approach to each pathology; LCTs require surgical treatment, while most cases of TARTs respond to medical treatment [4] [6]. To differentiate between the two syndromes, some features have been reported, even if there are no pathognomonic characteristics [19]. "
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    ABSTRACT: Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (LCTs) are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.
    02/2012; 2012:648643. DOI:10.1155/2012/648643
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    • "Based upon the histological appearance and clinical observations of TARTs, including natural history and available treatment options, a five stage classification of TARTs (Table 2) has recently been proposed by a group from the Netherlands with extensive clinical experience [69] [79] "
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    ABSTRACT: The treatment of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is complex. In addition to disease control, important therapeutic goals are the maintenance of normal growth and the acquisition of normal reproductive function. Here, data regarding final adult height (FH) in patients with CAH will be reviewed. Additional difficulties associated with CAH, including risks of obesity and hypertension, will be discussed. Information about fertility and reproductive outcomes in men and women with CAH will also be summarized. Although the treatment of each child with CAH needs to be individualized, close medical followup and laboratory monitoring along with good compliance can often result in positive clinical outcomes.
    International Journal of Pediatric Endocrinology 02/2010; 2010(1):298937. DOI:10.1155/2010/298937
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