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双侧睾丸网囊性发育异常伴肾发育不全。

Bilateral cystic dysplasia of the rete testis with renal adysplasia.

作者信息

Bouron-Dal Soglio Dorothée, Harvey Isabelle, Jovanovic Mubina, Oligny Luc L, Fournet Jean-Christophe

机构信息

Department of Pathology, Hôpital Sainte-Justine, 3175 chemin de la Côte-Ste-Catherine, Montréal, Québec H3T 1C5, Canada.

出版信息

Pediatr Dev Pathol. 2006 Mar-Apr;9(2):157-60. doi: 10.2350/08-05-0086.1. Epub 2006 Jun 16.

Abstract

Cystic dyplasia of the rete testis (CDRT) is an uncommon, generally unilateral lesion characterized by anastomosing cystic spaces lined by a flattened simple cuboidal epithelium in the rete testis. In the literature this lesion often is associated with an ipsilateral urogenital lesion such as renal agenesia or multicystic dysplasia of the kidney, in order of frequency. The pathogenesis is explained by some authors by their common embryologic origin. We are reporting the finding of bilateral CDRT associated with ultrasound-diagnosed renal adysplasia in a 20-week gestational age fetus with oligohydramnios. Although CDRT has been referred to as being associated with multicystic renal dysplasia or renal agenesis, the present case appears to be unique in combining all the malformations together.

摘要

睾丸网囊性发育异常(CDRT)是一种罕见的、通常为单侧的病变,其特征是睾丸网内有由扁平单层立方上皮衬里的相互吻合的囊腔。在文献中,这种病变常与同侧泌尿生殖系统病变相关,如肾缺如或多囊性肾发育不良,按发生频率排序。一些作者根据其共同的胚胎学起源来解释其发病机制。我们报告了一例孕20周、羊水过少的胎儿,经超声诊断为双侧CDRT并伴有肾发育不良。尽管CDRT已被认为与多囊性肾发育不良或肾缺如有关,但本病例似乎在将所有这些畸形合并在一起方面是独特的。

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