颅脑朗格汉斯细胞组织细胞增生症的影像学诊断  

Imaging Diagnosis of Langerhans Cell Histiocytosis in Skull and Brain

作　　者：袁小平[1] 潘恒[1] 黄穗乔[1] 

机构地区：[1]中山大学孙逸仙纪念医院放射科,广东广州510120

出　　处：《继续医学教育》2016年第5期132-133,共2页Continuing Medical Education

摘　　要：目的探讨颅脑朗格汉斯细胞组织细胞增生症的影像学诊断,以利于临床诊断。方法对我院32例颅脑朗格汉斯细胞组织细胞增生症患者的影像学表现进行回顾性分析,患者均有手术病理结果证实。结果 32例,其中发生颅骨21例,下丘脑、垂体柄8例,脑实质3例;32例,CT检查21例,MR检查25例,有CT及MR资料14例。年龄3~35岁,平均（16.8±1.03）岁;本病发生在颅脑有以下特点：（1）发病年龄轻,儿童或青少年多见;（2）病变颅骨骨破坏以骨板障为中心,破坏边缘清楚,少见坏死,发生在颞骨乳突区易误诊恶性肿瘤;（3）脑部常发生下丘脑、垂体柄,可见下丘脑、垂体柄增粗;偶见脑实质病变及脑神经变性改变。结论颅脑朗格汉斯细胞组织细胞增生症影像学表现有一定特征性,结合临床、病理,有利助于分析、诊断朗格汉斯细胞组织细胞增生症疾病。Objective To discuss imaging diagnosis of Langerhans cell histiocytosis（LCH） in skull and brain for definite clinical diagnosis. Methods A retrospective analysis was conducted in 32 cases which were confirmed by operation and pathology in our hospital. Results Among 32 cases, there were 21 cases with skull, 8 cases of with hypothaamus and stalk hypophysia., 3 cases with cerebral brain.32 cases,21 cases with CT, 25 cases with MR examination, both CT and MR data of 14 cases. Aged 3~35 years old, average（16.8±1.03） years. The disease occurs in skull and brain has the following characteristics：（1）The disease usually onset in children and adolescents.（2）The lesion skull bone destruction of bone diploe centered, destroy the edge is clear, rare necrosis, occur in the temporal bone mastoid area easily misdiagnosed as malignant tumor.（3）The brain often occur the hypothalamus, pituitary stalk, visible enlargement of the hypothalamus, pituitary stalk. Cranial nerve degeneration and the occasional parenchymal lesions are changing. Conclusions LCH in skull and brain have certain characteristic imaging findings, combined with the clinical, pathological, beneficial help to analysis and diagnosis the disease.

关 键 词：朗格汉斯细胞组织细胞增生症 影像诊断 颅脑 

分 类 号：R445[医药卫生—影像医学与核医学]