先天性肾上腺皮质增生症伴双侧睾丸肿瘤：一例报告。
Congenital adrenal hyperplasia presented with bilateral testicular tumor: A case report.
作者信息
Akbarzadeh Pasha Abazar, Shafi Hamid, Teimorian Mohamad, Rostami Ghasem, Nasirimehr Khatereh, Moudi Emadoddin
机构信息
Cancer Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran.
Clinical Research Development Center, Shahid Beheshti Hospital, Babol University of Medical Sciences, Babol, I.R.Iran.
出版信息
Caspian J Intern Med. 2021;12(Suppl 2):S431-S434. doi: 10.22088/cjim.12.0.431.
BACKGROUND
Congenital adrenal hyperplasia (CAH) refers to group of congenital diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21-hydroxylase deficiency. Testicular adrenal residual tumor (TART) is one of the major complications of CAH, possibly resulting from ectopic remnants of intra-testicular adrenal tissue which is stimulated by excessive secretion of adrenocorticotropic hormone (ACTH). This tumor can be misdiagnosed as Leydig cell tumor (LCT) in these people.
CASE PRESENTATION
The patient we are presenting is a 20-year-old man with a history of precocious puberty and a height below 3% of the population who underwent radical left testicular orchiectomy with a complaint of bilateral testicular mass, which is reported LCT in the pathology report. In preoperative imaging examinations, bilateral adrenal hyperplasia is observed. In hormonal examinations, the patient is diagnosed with CAH and has been treated with corticosteroids for one year.
CONCLUSION
In patients who present with bilateral testicular mass, it is the best image by abdominopelvic CT scan before surgery to detect CAH.
背景
先天性肾上腺皮质增生症（CAH）是指由于肾上腺类固醇生成受损导致的一组先天性疾病，其最常见的病因是21-羟化酶缺乏。睾丸肾上腺残余肿瘤（TART）是CAH的主要并发症之一，可能由睾丸内肾上腺组织的异位残余物引起，该组织受到促肾上腺皮质激素（ACTH）过度分泌的刺激。在这些患者中，这种肿瘤可能被误诊为睾丸间质细胞瘤（LCT）。
病例介绍
我们介绍的患者是一名20岁男性，有性早熟病史，身高低于人群的3%，因双侧睾丸肿块主诉接受了根治性左睾丸切除术，病理报告显示为LCT。在术前影像学检查中，观察到双侧肾上腺增生。在激素检查中，该患者被诊断为CAH，并已接受皮质类固醇治疗一年。
结论
对于出现双侧睾丸肿块的患者，术前进行腹部盆腔CT扫描是检测CAH的最佳影像学检查。
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