Testicular enlargement in patients with 11-hydroxylase deficiency

Karnak, İbrahìm; Şenocak, Mehmet Emìn; Göğüş, S; Büyükpamukçu, Nebìl; Hiçsönmez, Akgün

doi:10.1016/s0022-3468(97)90027-0

Cited by 25 publications

(23 citation statements)

References 11 publications

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“…Karnak e cols. (26) acompanharam um paciente com HRA bilateral por 16 meses, sob terapia supressiva com prednisolona 10 mg/dia, e observaram também grande redução do volume testicular e melhora da consistência, mas os achados ultrassonográficos de nódulos hipoecoicos persistiram. Por outro lado, há vários relatos de evolução com normalização do volume testicular e desaparecimento de nódulos, com o seguimento a longo prazo de tumores testiculares por HRA.…”

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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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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“…These tumors usually develop in untreated or inadequately treated boys. These tumors decrease in size with optimal steroid administration by suppression of the elevated ACTH levels [1][2][3].…”

Section: Discussionmentioning

confidence: 99%

Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia

et al. 2005

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The association between testicular tumors/nodules and congenital adrenal hyperplasia (CAH) has been recognized for many years. Tumors are considered to be an aberrant adrenal tissue that has descended with the testes and has become hyperplastic due to ACTH stimulation. The recommended treatment consists of increasing the glucocorticoid dose to suppress ACTH secretions. If the testicular size is not reduced after suppression therapy or a side effect of glucocorticoid dose is noted, surgical intervention should be considered. We diagnosed steroid unresponsive testicular tumors of the CAH in two patients who were treated by testicular sparing tumor enucleation. We believe that testis sparing surgery is the procedure of choice for all patients with testicular adrenal rest tumor, since it maximizes future fertility potential.

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“…Hyperplasia of testicular, ovarian and paragonadal rest tissue due to excessive ACTH stimulation is well recognized in adults and children with CAH [11, 14, 15, 16, 17, 18, 19, 20]. The incidence of hyperplasic adrenal rest tissue detected by ultrasound within the testis of patients with CAH is 24–27%.…”

Section: Discussionmentioning

confidence: 99%