Tumores testiculares bilaterais por hiperplasia congênita de restos adrenais

Fernandes, Virgínia Oliveira; Barros, A. I. S.; Quidute, Ana Rosa Pinto; Montenegro, Ana Paula Dias Rangel; Fontenele, Eveline Gadelha Pereira; Sales, Ana Paula Abreu Martins; Ferreira, Francisco Valdeci de Almeida; Montenegro, Renan Magalhães

doi:10.1590/s0004-27302009000800022

Cited by 3 publications

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References 25 publications

(40 reference statements)

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“…5 Recently, Fernandes et al reported a case of a 16-year-old boy who underwent bilateral orchiectomy because of TART that could not be differentiated from a malignant tumor, thus expressing this diagnostic dilemma. 54 Because of the rarity of TART and its management, we carried out a systematic analysis of the indexed articles published since 1966, in order to provide the best treatment for our patient.…”

Section: 38-43mentioning

confidence: 99%

Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

et al. 2011

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CONTEXT: Synthesis of cortisol and aldosterone is impaired in patients with congenital adrenal hyperplasia (CAH) because of 21-hydroxylase deficiency. Men with CAH have low fertility rates compared with the normal population, and this is related to testicular adrenal rest tumors. Findings of azoospermia in combination with a testicular tumor on ultrasound are likely to have a mechanical cause, especially when in the testicular mediastinum. The preferred treatment method consists of intensive corticoid therapy. However, when the tumor is unresponsive to steroid therapy, surgical treatment should be considered. CASE REPORT: We present the case of a male patient with CAH due to 21-hydroxylase deficiency who presented a testicular tumor and azoospermia. Treatment with low daily corticoid doses had previously been started by an endocrinologist, but after 12 months, no significant change in sperm count was found. Although the adrenocorticotrophic hormone and 17-hydroxyprogesterone levels returned to normal values, the follicle-stimulating hormone (FSH), luteinizing hormone and testosterone levels remained unchanged. Ultrasound examination confirmed that the testicles were small and heterogenous bilaterally, and revealed a mosaic area at the projection of the testis network bilaterally. Magnetic resonance imaging confirmed the finding. Testicular biopsy revealed the presence of preserved spermatogenesis and spermiogenesis in 20% of the seminiferous tubules in the right testicle. The patient underwent testis-sparing tumor resection. After 12 months of follow-up, there was no tumor recurrence but the patient still presented azoospermia and joined an intracytoplasmic sperm injection program.resUMo CONTEXTO: Pacientes com hiperplasia adrenal congênita (HAC) por deficiência da 21-hidroxilase podem ter a síntese de cortisol e de aldosterona prejudicada. Homens com HAC têm baixas taxas de fertilidade em comparação com a população normal, e isso está relacionado a tumores testiculares de remanescente adrenal. A associação de azoospermia e tumor testicular sugere uma causa mecânica, principalmente quando o tumor é encontrado no mediastino testicular. O método preferencial de tratamento consiste na corticoterapia intensa. No entanto, quando o tumor não é responsivo à terapia com esteroides, o tratamento cirúrgico deve ser considerado. RELATO DE CASO: Apresentamos o caso de um paciente do sexo masculino com HAC por deficiência da 21-hidroxilase, portador de tumor testicular e azoospermia. Em consulta prévia com endocrinologista, o paciente começou tratamento com baixas doses diárias de corticoide, porém, após 12 meses de tratamento, não houve mudança significativa no espermograma. Embora os níveis de hormônio adrenocortitrófico e 17-hidroxiprogesterona tenham se normalizado, os níveis séricos de hormônio folículo-estimulante, hormônio luteinizante e testosterona não se alteraram. Exame ultrassonográfico confirmou testículos bilateralmente diminuídos e heterogêneos, além de área em mosaico na projeção da rede testis bilate...

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Section: 38-43mentioning

confidence: 99%

Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

et al. 2011

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“…These tumors result from the failure in the migration of cells of the adrenal cortex, which have the same origin of primordial gonads. These cells migrate together and nestle within the gonadal tissue ( 1 , 3 - 5 ).…”

Section: Introductionmentioning

confidence: 99%

“…The presence of TART in a patient with CAH could lead to infertility due to direct compression of seminiferous tubules and/or paracrine toxicity from sexual steroids produced by TART ( 1 , 2 , 5 , 6 ). Serum hormonal levels of luteinizing hormone (LH), follicle-stimulant hormone (FSH), testosterone (T), and inhibin B could be impaired, therefore it is important to measure these hormonal levels to assess gonadal function of all patients with TART ( 7 , 9 ).…”

Section: Introductionmentioning

confidence: 99%

Testicular Adrenal Rests Tumors and Testicular Microlithiasis in a Brazilian Case Series with Classic Congenital Adrenal Hyperplasia

Carvalho

Lora

Penna

et al. 2016

Int J Endocrinol Metab

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BackgroundTesticular adrenal rest tumors are a benign condition characterized by the presence of remnants of adrenal tissue within the testes that can lead to infertility. Testicular microlithiasis are calculus deposits within the seminiferous tubules. Both are described in congenital adrenal hyperplasia.ObjectivesDescribe the frequency of testicular adrenal rest tumors and testicular microlithiasis in a Brazilian case series of patients with classic congenital adrenal hyperplasia and to also relate these changes to disease control and hypothalamic-pituitary-gonadal axis disorders.MethodsCase series study. An ultrasound examination of the scrotum was performed on 12 patients between the ages of 5.33 to 22 (14.72 ± 5.26) years. Testicular adrenal rest tumors were classified according to the degree of testicular infiltration in stages by adapting the Grinten’s classification, ranging from the absence of testicular adrenal rests visible by ultrasound (stage ≤ 1) to chronic obstruction of the testicular parenchyma with irreversible damage of the testicle (stage 5).ResultsSix patients (5 salt wasting and 1 simple virilizing) with an average age of 17.27 ± 3.09 years and have gone through puberty showed testicular adrenal rest tumors (Grinten stage ≥ 3). In 2 of the patients there was a coincidence with testicular microlithiasis. The frequency of testicular adrenal rest tumors did not relate with the levels of serum 17-hydroxyprogesterone and androstenedione. In 3 patients with testicular adrenal rest tumors, gonadotropin levels were suggestive of hypergonadotropic hypogonadism and one of hypogonadotropic hypogonadism.ConclusionsTesticular adrenal rest tumors were found in greater frequency during puberty and was not related to hormonal control in this group. Some of them happened with testicular microlithiasis.

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

Werneck

Rodrigues

Mantovani

et al. 2019

Journal of Pediatric Endocrinology and Metabolism

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Background Testicular 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. Methods Twelve 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. Results The 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). Conclusions We 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.

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Tumores testiculares bilaterais por hiperplasia congênita de restos adrenais

Cited by 3 publications

References 25 publications

Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

Testicular Adrenal Rests Tumors and Testicular Microlithiasis in a Brazilian Case Series with Classic Congenital Adrenal Hyperplasia

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

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