儿童丘脑Rosai-Dorfman病1例并文献复习

Thalamic Rosai-Dorfman disease in children:a case report and literature review

目的探讨儿童丘脑Rosai-Dorfman病(RDD)的临床表现、病理特征和影像特点,以提高对该病的影像诊断和鉴别诊断能力。方法回顾性分析1例经手术病理证实为丘脑RDD的临床病理特征、MRI表现,并复习文献予以总结。结果MRI显示右侧丘脑类圆形肿物,T1WI、T2WI和液体衰减反转恢复(FLAIR)序列上均呈低信号,信号均匀;增强扫描上病灶明显强化,边缘光滑,邻近脑组织受压水肿。结论儿童丘脑RDD罕见,其临床和影像表现无明显特异性,确诊需要依靠病理组织学检查。

Objective To investigate the clinical,pathological and imaging features of thalamic Rosai-Dorfman disease(RDD)in children,so as to improve the ability of imaging diagnosis and differential diagnosis.Methods The clinicopathological features and MRI manifestations of 1 thalamic RDD case confirmed by surgery and pathology were retrospectively analyzed,and the literature was reviewed and summarized.Results MRI showed a round mass in the right thalamus.The mass showed homogenerous hypointensity on T1WI,T2WI,and fluid attenuated inversion recovery(FLAIR),and was significantly enhanced with smooth margin on enhanced scanning.The adjacent brain tissue was compressed and edematous.Conclusion Thalamic RDD in children is rare,and its clinical and imaging findings are not significantly specific.The diagnosis requires histopathological examination.