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睾丸网囊性发育异常。病例报告。

Cystic dysplasia of the rete testis. Case report.

作者信息

Kajo K, Matoska J, Javorka K, Macháleková K, Tomaskin R, Kliment K

机构信息

Department of Pathology, Jessenius Faculty of Medicine, Comenius University, Martin, Slovakia.

出版信息

APMIS. 2005 Oct;113(10):720-3. doi: 10.1111/j.1600-0463.2005.apm_342.x.

Abstract

Cystic dysplasia of the rete testis (CDRT) is a very rare cause of a paediatric scrotal mass often associated with renal and other genitourinary tract anomalies. These complex malformations are probably due to a developmental defect of the mesometanephric system during embryogenesis. A case of asymptomatic scrotal swelling in a 4-year-old boy is presented. Ultrasonography, showed a cystic lesion of the left testis associated with absence of the left kidney. Orchiectomy was performed because of extensive gonad involvement. Pathologic examination revealed multiple, anastomosing, irregular cystic spaces of varying sizes and shapes predominantly located in the region of the rete testis. The cysts had spread irregularly, displacing the testicular parenchyma, which was subsequently compressed under the tunica albuginea. Preoperative diagnosis of CDRT is easy if age, precise localisation, characteristic ultrasonographic features and other genitourinary malformations are considered. Other paediatric cystic lesions should be included in the differential diagnosis. It is possible to cure CDRT by orchiectomy or by conservative treatment. Nowadays the later option is preferred, but diagnosis of CDRT must be precisely established and followed by careful monitoring.

摘要

睾丸网囊性发育异常(CDRT)是小儿阴囊肿块的一种非常罕见的病因,常与肾脏及其他泌尿生殖道异常相关。这些复杂的畸形可能是由于胚胎发育过程中中后肾系统的发育缺陷所致。本文报告一例4岁男孩无症状阴囊肿胀的病例。超声检查显示左侧睾丸有一个囊性病变,同时左侧肾脏缺如。由于性腺广泛受累,遂行睾丸切除术。病理检查发现多个大小和形状各异的、相互吻合的不规则囊性间隙,主要位于睾丸网区域。囊肿呈不规则扩散,推移睾丸实质,随后睾丸实质被白膜压迫。如果考虑患者年龄、精确的定位、特征性的超声表现及其他泌尿生殖系统畸形,CDRT的术前诊断并不困难。鉴别诊断应包括其他小儿囊性病变。CDRT可通过睾丸切除术或保守治疗治愈。如今更倾向于后一种选择,但必须准确诊断CDRT并进行仔细监测。

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