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

Grinten, Hedi L Claahsen–van der; Otten, Barto J.; Hermus, A.R.M.M.; Sweep, Fred C.G.J.; Kaa, Christina A. Hulsbergen‐van de

doi:10.1016/j.fertnstert.2007.03.051

Cited by 119 publications

(110 citation statements)

References 29 publications

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“…Therefore, there seems to be no need to treat or remove the tumours at an early stage. However, because of the localisation of the tumours in the rete testis, the tumours may compress the seminiferous tubules leading to obstructive azoospermia and irreversible damage of the testes (2,25). Therefore, it is important to detect and treat the tumours before permanent damage of the testis has occurred.…”

Section: Discussionmentioning

confidence: 99%

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Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Grinten¹,

Sweep²,

Blickman³

et al. 2007

eur j endocrinol

133

117

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Objective: Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction of the seminiferous tubules. The aim of our study was to determine the presence of TART and their influence on gonadal function in childhood. Design: Retrospective study. Patients and methods: Scrotal ultrasound was performed in 34 children with CAH due to 21-hydroxylase deficiency who were between 2 and 18 years old. FSH, LH, testosterone and inhibin B concentrations were measured in serum of 27 patients. Results: TART were detected by ultrasound in 8 out of 34 (24%) children. In two of them, bilateral tumours were found. All lesions were located in the rete testis. Seven patients had the salt-wasting type of CAH; one patient had the simple virilising type of CAH. Mean tumour size was 4.1 mm (range 2-8 mm). In none of the patients were the tumours palpable. Two children with TART were between 5 and 10 years old, the other six children were above 10 years old. In all children with TART, LH, FSH, testosterone and inhibin B levels were similar to the patients without TART. Conclusion: TART can be found in CAH children before the age of 10 years. The absence of gonadal dysfunction in our group of children suggests that gonadal dysfunction as frequently reported in adult CAH patients with TART develops after childhood.

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Section: Discussionmentioning

confidence: 99%

“…The tumours are always benign. However, because of their typical location within the rete testis, the tumours may lead to obstruction of the seminiferous tubules with possible gonadal dysfunction and infertility (2). Therefore, it may be important to detect and treat the tumours in an early stage.…”

Section: Introductionmentioning

confidence: 99%

Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Grinten¹,

Sweep²,

Blickman³

et al. 2007

eur j endocrinol

133

117

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“…Due to their central location, TARTs can compress the seminiferous tubules and cause end-stage damage of the testicular parenchyma [72]. Testicular biopsies of 7 male patients with long-standing TARTs revealed decreased spermatogenesis and decreased tubular diameter with peritubular fibrosis [72].…”

Section: Reproductive Outcome—menmentioning

confidence: 99%

Growth and Reproductive Outcomes in Congenital Adrenal Hyperplasia

Nebesio

Eugster

2010

International Journal of Pediatric Endocrinology

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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.

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“…A localização central do tumor perto do mediastino testicular, causando compressão dos túbulos seminíferos, poderia levar à azoospermia obstrutiva e a danos irreversíveis ao tecido testicular adjacente (8). Um recente estudo demonstrou uma diminuição de diâme-tro tubular e um grau variável de fibrose peritubular e hialinização tubular na biópsia testicular, além de clínica de infertilidade em sete pacientes com HAC e HRA bilateral de longa data (16). Além disso, foi observada diminuição acentuada do número de células germinativas em todos os pacientes (8).…”

Section: Discussionunclassified

“…Assim, nessa fase mais avançada de comprometimento, a única indicação para a cirurgia é o alívio da dor e do desconforto causado pela HRA (8). No caso de pacientes inférteis, a biópsia testicular pode ser útil para avaliar a viabilidade e a qualidade do parênqui-ma testicular residual (16).…”

Section: Discussionunclassified

Tumores testiculares bilaterais por hiperplasia congênita de restos adrenais

Fernandes

Barros

Quidute

et al. 2009

Arq Bras Endocrinol Metab

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RESUMOObjetivos: Tumores testiculares são uma rara condição associada à hiperplasia adrenal congê-nita (HAC) que decorrem da hiperplasia de restos adrenais intratesticulares (HRA), raramente ocorrendo associados a neoplasias malignas. Sua diferenciação histológica com tumores de cé-lulas de Leydig é muito difícil, podendo levar a orquiectomias desnecessárias. O objetivo deste relato foi apresentar esse dilema diagnóstico em um paciente com HAC e tumores testiculares bilaterais. Métodos: Relatou-se o caso de um paciente masculino, 16 anos, com diagnóstico de HAC desde os 3 anos de idade, que apresentava tumorações testiculares endurecidas, indolores e de crescimento lento, sendo encaminhado para orquiectomia bilateral. Resultados: Foi decidido por tratamento conservador com prednisona, havendo significativa diminuição do volume testicular e normalização dos níveis de andrógenos. Conclusão: Este caso demonstra a importância de sempre se considerar a hipótese de HRA intratesticulares no diagnóstico diferencial dos tumores testiculares. A investigação e a conduta devem ser conduzidas de maneira cautelosa para se evitar orquiectomias desnecessárias. Arq Bras Endocrinol Metab. 2009;53(8):1052-8 Descritores Hiperplasia adrenal congênita; tumor testicular; hiperplasia de restos adrenais intratesticulares ABSTRACT Objectives: Testicular tumors are a rare condition associated with congenital adrenal hyperplasia (CAH), originated from intratesticular adrenal rest tumors, and they are rarely associated with malignant tumors. Their histological differentiation from Leydig-cell tumors is quite difficult, which would lead to inappropriate orchiectomies. Thus the objective of this report was to present this diagnostic dilemma. Methods: Reported the case of 16-yr-old boy with previous diagnosis of CAH with bilateral testicular enlargement who was recommended to be submitted to a bilateral orchiectomy. Results: Considering this findings, it was decided to treat conventionally with prednisone with significant reduction of testicular volume, and normalization of androgens levels. Conclusion: This case shows the importance of intratesticular adrenal rest tumors in the differential diagnosis of testicular tumors. Cautious approach during investigation and treatment are recommended to avoid inappropriate orchiectomies.

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

Cited by 119 publications

References 29 publications

Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Growth and Reproductive Outcomes in Congenital Adrenal Hyperplasia

Tumores testiculares bilaterais por hiperplasia congênita de restos adrenais

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