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Journals / Gülhane Tıp Dergisi / 2013 / Cilt: 55 - Sayı: 1

A Case of congenital adrenal hyperplasia with testicular and intraabdominal adrenal rest tumor presented with testicular mass

Testislerde kitle ile başvuran testiküler ve intraabdominal adrenal rest tümörlü bir konjenital adrenal hiperplazi olgusu

Metin ALIŞ
LEVENT ÖZSARI
Gökhan ÖZIŞIK
Sinan ÇAĞLAYAN
M. Emin ÖNDE
Pages
42–45
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

After steroid replacement therapy, bilateral orchiectomy and unilateral adrenal mass excition performed.

📌 3 Key Takeaways

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Objective & Hypothesis:Congenital adrenal hyperplasia (CAH) is a disease that occurs with enzyme defect in steroid synthesis. 21-hydroxylase enzyme deficiency is presented in over 90 per cent of the cases. 21-hydroxylase deficiency has three different clinical forms which are salt wasted, simple virilizing and non-classical.
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Key Findings & Data:Because of the enzyme deficiency, steroid synthesis decreases and adrenocorticotropic hormon release increases.
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Conclusion & Impact:After steroid replacement therapy, bilateral orchiectomy and unilateral adrenal mass excition performed.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Congenital adrenal hyperplasia (CAH) is a disease that occurs with enzyme defect in steroid synthesis. 21-hydroxylase enzyme deficiency is presented in over 90 per cent of the cases. 21-hydroxylase deficiency has three different clinical forms which are salt wasted, simple virilizing and non-classical. Because of the enzyme deficiency, steroid synthesis decreases and adrenocorticotropic hormon release increases. All adrenal cortical cells increase. % 8.2 of the male patients with CAH have testicular mass. It is presented in patients with no treatment and generally bilateral. Because of the morphological and functional similarity with adrenal tissue, it is called testicular adrenal rest tumor (TART). A 20 year-old man presented with bilateral testicular masses. His history is accorded with puberty precocity. Laboratory tests showed hypoglycemia without symptoms. Further laboratory and imaging tests showed primary adrenal insufficiency , bilateral adrenal hyperplasia, intraabdominal and testicular masses. After steroid replacement therapy, bilateral orchiectomy and unilateral adrenal mass excition performed.

Özet

Konjenital adrenal hiperplazi (KAH) steroid sentezindeki bozukluk sonucu oluşur. Olguların % 90’ından fazlasında 21-hidroksilaz enzim eksikliği vardır. 21-hidroksilaz eksikliğinin üç faklı klinik formu vardır. Bunlar tuz kaybettiren, basit virilizan ve klasik olmayan tiplerdir. Enzim eksikliği nedeni ile steroid sentezi azalır ve adrenokortikotropik hormon (ACTH) düzeyi artar. Tüm adrenal korteks kaynaklı hücreler çoğalır. KAH’lı erkek hastaların % 8.2’sinde testiküler tümör bulunur. Genellikle bilateraldir ve tedavi almayan hastalarda görülür. Fonksiyonel ve morfolojik olarak adrenal dokuya benzediği için testiküler adrenal rest tümör (TART) olarak isimlendirilir. 20 yaşında erkek hasta bilateral testiküler kitle ile başvurdu. Özgeçmişi puberte prekoks ile uyumlu olarak değerlendirildi. Yapılan tetkikleri sonucu semptomatik olmayan hipoglisemi saptandı. Yapılan ileri tetkikleri sonucu primer adrenal yetersizlik ve bilateral adrenal hiperplazi , intraabdominal ve testiküler kitleler saptandı. Steroid replasman tedavisi sonrası bilateral orşiektomi ve unilateral adrenalektomi ve adrenal bez komşuluğundaki kitle eksizyonu operasyonları uygulandı.

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Cite This Article

ALIŞ, M., ÖZSARI, L., ÖZIŞIK, G., ÇAĞLAYAN, S., ÖNDE, M. E. (2013). A Case of congenital adrenal hyperplasia with testicular and intraabdominal adrenal rest tumor presented with testicular mass. Gülhane Tıp Dergisi, Cilt: 55 - Sayı: 1, 42–45. https://ajindex.com/articles/225317

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References (8)

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