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一名先天性肾上腺皮质增生症患者的大型睾丸肾上腺残余肿瘤。

Large testicular adrenal rest tumours in a patient with congenital adrenal hyperplasia.

作者信息

Rajkanna J, Oyibo S O

机构信息

Department of Endocrinology, Peterborough City Hospital Bretton Gate , Peterborough PE3 9GZ , UK.

出版信息

Endocrinol Diabetes Metab Case Rep. 2015;2015:140080. doi: 10.1530/EDM-14-0080. Epub 2015 Feb 1.

DOI:10.1530/EDM-14-0080
PMID:25755878
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4315946/
Abstract

UNLABELLED

Testicular adrenal rest tumours (TARTs) are benign ACTH-dependent tumours that occur in males with congenital adrenal hyperplasia (CAH) and if left untreated can destroy testicular tissue. Corticosteroid suppressive treatment could result in the regression of these testicular tumours. We present a patient with bilateral large TARTs as a consequence of poor compliance to treatment and follow-up for his CAH, who consequently had to have bilateral orchidectomies and prosthesis replacement.

LEARNING POINTS

TARTs are frequently seen in males with CAH, and can be misdiagnosed as primary testicular cancer.Patient compliance to treatment and follow-up are necessary to reduce the risk of testicular damage as a result of TARTs in patients with CAH.Boys with CAH should have periodic ultrasonographic screening from before adolescent age for early detection of TARTs.Regular monitoring of renin, 17-hydroxyprogesterone and androgens levels is required to assess corticosteroid suppressive treatment.Patients with CAH should be offered psychological support and information concerning CAH support groups.

摘要

未标注

睾丸肾上腺残余肿瘤(TARTs)是一种良性的促肾上腺皮质激素(ACTH)依赖性肿瘤,发生于患有先天性肾上腺皮质增生症(CAH)的男性患者中,若不治疗可破坏睾丸组织。皮质类固醇抑制治疗可使这些睾丸肿瘤消退。我们报告一例因对其CAH治疗和随访依从性差而出现双侧巨大TARTs的患者,因此不得不接受双侧睾丸切除术及假体置换。

学习要点

TARTs在患有CAH的男性中常见,可能被误诊为原发性睾丸癌。患者对治疗和随访的依从性对于降低CAH患者因TARTs导致睾丸损伤的风险很有必要。患有CAH的男孩应在青春期前定期进行超声筛查,以便早期发现TARTs。需要定期监测肾素、17-羟孕酮和雄激素水平以评估皮质类固醇抑制治疗效果。应为CAH患者提供心理支持以及有关CAH支持小组的信息。

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本文引用的文献

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Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency.11β-羟化酶缺乏所致先天性肾上腺皮质增生症患者中与睾丸肾上腺残余肿瘤相关的睾丸间质细胞瘤
Case Rep Urol. 2012;2012:648643. doi: 10.1155/2012/648643. Epub 2012 Feb 12.
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Subjective health status in men and women with congenital adrenal hyperplasia: a population-based survey in Norway.男性和女性先天性肾上腺皮质增生患者的主观健康状况:挪威的一项基于人群的调查。
Eur J Endocrinol. 2010 Sep;163(3):453-9. doi: 10.1530/EJE-10-0284. Epub 2010 Jun 15.
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Testicular adrenal rest tumours in congenital adrenal hyperplasia.先天性肾上腺皮质增生症中的睾丸肾上腺残余肿瘤
Int J Pediatr Endocrinol. 2009;2009:624823. doi: 10.1155/2009/624823. Epub 2009 Feb 26.
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Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症男性患儿睾丸肾上腺残余瘤的患病率
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Congenital adrenal hyperplasia in adults: a review of medical, surgical and psychological issues.成人先天性肾上腺增生症:医学、外科及心理问题综述
Clin Endocrinol (Oxf). 2006 Jan;64(1):2-11. doi: 10.1111/j.1365-2265.2005.02410.x.
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Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia.先天性肾上腺皮质增生症类固醇无反应性睾丸肿瘤的保留睾丸手术
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