Erdheim-Chester病泌尿系统受累CT影像学表现

CT imaging findings of urinary system involvement in Erdheim-Chesterdisease

目的:Erdheim-Chester病(ECD)诊断依赖临床、影像及组织病理学结合。本文主要探讨ECD患者泌尿系统受累CT影像学特征。方法:分析北京协和医院2014年1月-2019年9月确诊ECD 41例患者(男25例,女16例;年龄4~63岁,39.2±17.1岁)腹盆部CT图像,研究其泌尿系统影像学特征,包括病灶形态、密度、累及部位及强化特点等。结果:41例患者21例累及泌尿系统。腹部CT示21例患者肾脏受累,15例患者输尿管受累,8例患者肾动脉受累。2例患者临床表现尿崩症,11例患者肾积水。本研究14例(14/21)ECD患者发现特有肾周软组织浸润影像学表现,呈典型“卷发征”。结论:泌尿系统是ECD患者常累及系统之一,对于腹部CT出现肾周软组织浸润患者即使未出现泌尿系统相关临床症状也应考虑Erdheim-Chester病。

Objective:The diagnosis of Erdheim-Chester disease depends on the combination of clinical,imaging and histopathology.This paper mainly discusses the CT imaging features of urinary system involvement in patients with ECD.Methods:The abdomen CT images 41 ECD patients(25 males,16 females;aged 4-63 years,37.2±17.1 years)in Peking Union Medical College Hospital from January 2014 to September 2019 were analyzed retrospectively,and the imaging features of urinary system,including the shape,density,involved site and enhancement characteristics of the lesions,were studied.Results:Twenty-one patients were involved the urinary system.Abdominal CT showed renal involvement in 21 patients,ureter involvement in 15 patients,and renal artery involvement in 8 patients.The clinical manifestations of 2 patients were diabetes insipidus and 11 patients were hydronephrosis.The unique imaging findings of perirenal soft tissue infiltration were found in 14(14/21,66.7%)patients with ECD,which showed a typical"hairy kidney"sign.Conclusion:Urinary system is one of the most common systems in patients with ECD.For patients with perirenal soft tissue infiltration in abdominal CT,Erdheim-chester disease should be considered even if there are no clinical symptoms related to urinary system.