先天性肾上腺增生或睾丸间质细胞肿瘤患者的双侧同步睾丸肿块?是否双侧睾丸切除术的困境

Christos Leventis, P. Panagopoulos, E. Delliou, A. Syrnioti, V. Papamichail
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引用次数: 0

摘要

先天性肾上腺增生症(先天性肾上腺增生症)是指由于合成类固醇激素的酶缺乏而引起的一组自主神经紊乱。这些疾病导致ACTH水平升高,随后出现肾上腺增生。CAH分为经典型和非经典型两种。在普通类型中,我们观察到21-羟化酶缺乏症的患病率为每145,000名新生儿中每5000人中有1人。我们的醛固酮和皮质醇分泌不足,结果是血浆ACTH水平升高,这导致了随后的疾病,这取决于缺乏的程度。睾丸肾上腺休息肿瘤(TART)发生于性腺内的胰岛切分肾上腺组织,直接受ACTH过量产生(CAH并发症)的影响,患病率为27%至47%。这些是可触及肿块的良性肿瘤。建议对这些肿瘤进行活检和手术切除,以排除恶性肿瘤。在这个病例报告中,我们提出了一例伴有CAH患者的双侧同步TART肿瘤合并髓质脂肪瘤的病例。关于双侧睾丸切除术的决定出现了困境。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Bilateral synchronous testicular mass from testicular adrenal rest tumors, in a patient with congenital adrenal hyperplasia, or testicular leydig cell tumors? Dilemma for bilateral orchiectomy or not
Congenital adrenal hyperplasia (CAH) refers to a group of autonomic disorders due to enzyme deficiency for the biosynthesis of steroid hormones. These disorders entail an increase in ACTH levels and as followed by adrenal hyperplasia. CAH is categorized into two types, classic and non-classic. In the common type we have a deficiency of 21-hydroxylase observed in a prevalence of 1 per 5000 per 145,000 births. We have insufficient aldosterone and cortisol production and as a result, elevated plasma ACTH levels, with subsequent disorders that this entails, depending on the level of deficiency. Testicular adrenal rest tumor (TART) develops from isletsectopic adrenal tissue within the gonads, directly affected by ACTH overproductions a complication of CAH, with a prevalence ranging from 27% to 47%. These are benign tumors that are recognized as palpable masses. A biopsy of these tumors is recommended as well as their surgical removal, so as to rule out malignancy.In this case report we present a case of bilateral synchronous TART tumor in association with medullolipoma in a patient with CAH. The dilemma arises regarding the decision of bilateral orchiectomy.
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