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睾丸退化综合征的组织病理学特征:与患者年龄的关系及对治疗的影响

Histopathological features of testicular regression syndrome: relation to patient age and implications for management.

作者信息

Law H, Mushtaq I, Wingrove K, Malone M, Sebire N J

机构信息

Department of Histopathology, Great Ormond Street Hospital, London, UK.

出版信息

Fetal Pediatr Pathol. 2006 Mar-Apr;25(2):119-29. doi: 10.1080/15513810600788806.

Abstract

Testicular regression syndrome (TRS) represents a congenital condition in which no normal testicular tissue can be identified following exploration for a clinically impalpable testis. A spectrum of pathological findings may be present but there is little literature systematically examining these features. We searched a pediatric histopathology database to identify cases of TRS, and the histopathological findings were reviewed and pooled with those of all previously published smaller series. A total of 117 cases were identified during the period (1989-2004), median age 2 (range birth-12) years. In 52 (44%) a nodule was identified macroscopically, median maximum diameter 0.5 (range 0.1-2.0) cm. Microscopic hemosiderin-laden macrophages were present in 85 (73%), dystrophic calcification in 52 (44%), residual testicular tubules in 12 (10%), vas deferens in 71 (61%), and epididymal tissue in 39 (33%). The prevalence of hemosiderin laden macrophages and dystrophic calcification were significantly greater in cases < or =3 years (84% versus 64% and 55% versus 32%, respectively). But there was no significant difference in the frequency of other findings between the younger and older age groups; in particular, the presence of residual testicular tubules was similar (7% versus 13%, respectively). Furthermore, there was no significant correlation between identification of a macroscopically distinct nodule and presence of residual tubular structures, tubules being identified in 6 of the 65 cases in which no clearly identifiable nodule was seen macroscopically. The presence of hemosiderin-laden macrophages and foci of dystrophic calcification showed a positive association. TRS is associated with specific histopathological features, the findings being consistent with changes secondary to intrauterine testicular torsion. Residual testicular tubules are found in 10% of cases regardless of the presence or absence of a macroscopically identifiable nodule.

摘要

睾丸退化综合征(TRS)是一种先天性疾病,在对临床无法触及的睾丸进行探查后,无法发现正常的睾丸组织。可能存在一系列病理表现,但很少有文献系统地研究这些特征。我们检索了一个儿科组织病理学数据库以识别TRS病例,并对组织病理学结果进行了回顾,并与之前所有已发表的较小系列研究的结果进行汇总。在此期间(1989 - 2004年)共识别出117例病例,中位年龄为2岁(范围从出生至12岁)。52例(44%)在大体检查时发现有结节,中位最大直径为0.5厘米(范围0.1 - 2.0厘米)。85例(73%)显微镜下可见含铁血黄素巨噬细胞,52例(44%)有营养不良性钙化,12例(10%)有残留的睾丸小管,71例(61%)有输精管,39例(33%)有附睾组织。年龄≤3岁的病例中,含铁血黄素巨噬细胞和营养不良性钙化的发生率显著更高(分别为84%对64%和55%对32%)。但在较年轻和较年长年龄组之间,其他发现的频率没有显著差异;特别是残留睾丸小管的存在情况相似(分别为7%对13%)。此外,大体上明显的结节识别与残留管状结构的存在之间没有显著相关性,在65例大体上未见明显可识别结节的病例中,有6例发现了小管。含铁血黄素巨噬细胞的存在与营养不良性钙化灶呈正相关。TRS与特定的组织病理学特征相关,这些发现与宫内睾丸扭转继发的变化一致。无论是否存在大体上可识别的结节,10%的病例中可发现残留的睾丸小管。

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